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Download MBBS Biochemistry PPT 44 Final Biologcal Oxidation 18 Lecture Notes

Download MBBS (Bachelor of Medicine, Bachelor of Surgery) 1st year (First Year) Biochemistry ppt lectures Topic 44 Final Biologcal Oxidation 18 Notes. - biochemistry notes pdf, biochemistry mbbs 1st year notes pdf, biochemistry mbbs notes pdf, biochemistry lecture notes, paramedical biochemistry notes, medical biochemistry pdf, biochemistry lecture notes 2022 ppt, biochemistry pdf.

This post was last modified on 05 April 2022

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Energy Metabolism

Bioenergetics

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BIOLOGICAL OXIDATION
Specific Learning Objectives

Questions Which Wil be Answered

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What is system of Bioenergetics ?

How is chemical form of energy ATP formed (Generation)
and utilized (Operation) in human body ?

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What Factors are associated to bioenergetics system?

Metabolites
Enzymes
Coenzymes

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Cofactors
Hormones

Which disorders suffered due to defective system ?
Synopsis

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vWhat is Bioenergetics?
vHigh Energy Compounds
vSubstrate Level Phosphorylation
vWhat is Biological Oxidation?

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vEnzymes and Coenzymes of Biological

Oxidation Reactions

vElectron Transport Chain (ETC)

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Continued---------

vOxidative Phosphorylation Mechanism
vInhibitors of ETC and Oxidative Phosphorylation

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vUncouplers- Mode of Action

vShuttle System

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vFactors Involved in Oxidative Phosphorylation

mechanism


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Lets Get Introduced To
What Is Bioenergetics?

Bioenergetics or biochemical

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thermodynamics is:

Study of energy changes during

biochemical reactions.

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Biological Systems Conform to General

Laws of Thermodynamics

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Energy Is Never Destructed

(Soul is energy never destructed and it is Immortal)

Total energy of a system, including its surroundings,

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remains constant

Energy is neither lost nor gained during any change

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May be transformed into another form of energy

May be transferred from one part of system to another

or

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Conditions Of Bioenergetics

Isothermic (mostly)

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Endothermic/

Endergonic/Anabolic

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Exothermic/Exergonic

/Catabolic

ENDERGONIC ( Anabolic) PROCESSES

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PROCEED BY COUPLING

OF EXERGONIC(Catabolic)

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PROCESSES
High Energy Compounds Of

Human Body

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High energy compounds are energy

rich compounds.

Possess high energy bonds in its

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structures.

Cleavage of these high energy bonds

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liberate more energy than that of

ATP hydrolysis.
S.No

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Examples Of High

Free Energy

Energy Compounds

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Released On

Hydrolysis.

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Cal/mol

1

Phospho Enol Pyruvate

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-14.8

2

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Carbamoyl Phosphate

- 12.3

3

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Cyclic AMP

-12.0

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4

1,3 Bis Phospho Glycerate

-11.8

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S.No

Examples Of High Energy

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Free Energy

Compounds

Released On

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Hydrolysis.

Cal/mol

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5

Creatine Phosphate

-10.3

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6

S Adenosine Methionine

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-10.0

( SAM)

7

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Succinyl CoA

-7.7

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8

Acetyl CoA

-7.7

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9

ATP

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-7.3


Significance Of High Energy

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Compounds

OR

Fates Of High Energy Compound

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In Catabolic And Anabolic

Pathways
During Catabolic pathways/reaction

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High energy compounds follow

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substrate level phosphorylation

reaction.

High energy compounds cleave high

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energy bond to generate high energy

used for phosphorylation of ADP with

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pi at reaction level.

Generate ATP at substrate/reaction

level.

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Substrate Level Phosphorylation

Mode of generation of ATP at substrate level

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Involves cleavage of high energy bond

present in high energy compound

Bond energy released is used for

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Phosphorylation reaction

Generates ATP directly and instantly at

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reaction level without involvement of ETC
Examples Of High Energy

Compounds Undergoing Substrate

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Level Phosphorylation.

S.No

High Energy

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Enzyme

Product

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High

Metabolic

Compound

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Catalyzing

Obtained

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energy

Pathway

Phosphate

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Involved

Compound

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Generated

1

1,3 Bis

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Phospho

3

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ATP

Glycolysis

Phospho

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Glycerate Phospho

Glycerate

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Kinase

Glycerate

2

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Phospho

Pyruvate

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Enol

ATP

Glycolysis

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Enol

Kinase

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Pyruvate

Pyruvate

3

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Succinyl

Succinate Succinate

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GTP

Krebs/TCA

CoA

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Thio

Cycle

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Kinase
During Anabolic pathways/reaction

High energy compounds follow

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condensation or bond building

reactions.

High energy compound cleave to

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generate energy

Energy used for building C-C bonds.

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HIGH-ENERGY PHOSPHATES

PLAY A CENTRAL ROLE IN ENERGY

CAPTURE AND TRANSFER

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High Energy Compounds Generated In

Catabolic Pathways Are Utilized In

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Anabolic Reactions

HIGH-ENERGY PHOSPHATES

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ACT AS

"ENERGY CURRENCY" OF CELL


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Free Energy of hydrolysis Of High

Energy Phosphate Bonds has

Important Bioenergetics

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Significance

Adenylate Kinase (Myokinase)

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Interconverts Adenine Nucleotides
Important Features Of ATP

Contains three high energy phosphate bonds

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Drive endergonic reactions

It is chemical energy currency of body

Functions in body as a complex with Mg2+

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Biosynthesized by ATP synthase

Couples thermodynamically Unfavorable reactions to Favorable Ones

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ATP synthesis is inhibited by Uncouplers

What Is Biological Oxidation?
Biological oxidations :

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Oxidation

reactions/Process

Occurring in living

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cells.

Importance/Features Of

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Biological Oxidation
Biological Oxidation Reactions/Process :

Involves Oxygen

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Associated with metabolism

Generates ATP

Vital for functioning of cells

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Survival and existence of human

body.

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Definition Of Oxidation Reactions
Oxidation reactions are

biochemical reactions where there is

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either:

Removal / Loss of Hydrogen
(Dehydrogenation)
Removal or Loss of Electrons

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Addition of Oxygen
(Oxygenation)

Feature Of Biological Oxidation

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Oxidation of a molecule (electron

donor) is always accompanied by

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reduction of a second molecule

(electron acceptor)
Most predominant type of

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Oxidation reaction in body

is:

Dehydrogenation Reaction

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Catalyzed by Dehydrogenases

Dehydrogenases catalyzes

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to remove Hydrogen from

substrates.

Which are temporarily

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accepted by Coenzymes.
Coenzymes

and

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Enzymes

of

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Biological Oxidation

Reactions

Coenzymes

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and

Inorganic Cofactors

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Of

Biological Oxidation

Reactions

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FMN
FAD
NAD+
NADP+
THBP (Tetra Hydro Biopterin)

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Cu++
Fe+++

Oxidized Coenzymes involved in

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Oxidation/Dehydrogenation

reactions.

NAD+

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NADP+

FAD

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FMN
Oxidized Coenzymes temporarily accept

the hydrogen from substrates and get

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transformed to reduced coenzymes.

NADH+H+

FADH2

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NADPH+H+

FMNH2

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The reduced and oxidized forms of NAD


The reduced and oxidized forms of FAD

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5 Enzymes of Biological Oxidation
1. AEROBIC

DEHYDROGENASES

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2. ANAEROBIC

DEHYDROGENASES

3. OXYGENASES

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4. OXIDASES
5. HYDROPEROXIDASES

All 5 Enzymes of

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Biological Oxidation

reactions are classified

in

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Class I

Oxido Reductases
AEROBIC DEHYDROGENASES

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Aerobic Dehydrogenases are Flavoproteins
Enzymes covalently bound to coenzymes FMN or

FAD

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MH2 Aerobic DH M

FMN FMNH2 (Auto

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oxidizable)

Catalase

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H2 +O2 H2O2 H2O + O2
FMN/FAD are acceptors

of removed Hydrogen

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Reduced Coenzymes

(FMNH2/FADH2) formed

are auto oxidizable

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Reduced coenzymes get

reoxidized at reaction

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level.

Oxygen gets directly

involved at reaction level to

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reoxidize the reduced

coenzymes.
H2O2 is a byproduct of

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Aerobic Dehyrogenase

activity.

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Catalase then detoxify

the H2O2 to H2O and O2.

Specific Examples Of

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Aerobic Dehydrogenases

L Amino acid Oxidase
(Oxidative Deamination of A.A)

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Xanthine Oxidase
(Purine Catabolism)
Glucose Oxidase
(Glucose Oxidation to Gluconic acid)
Aldehyde Dehydrogenase

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( Alcohol Metabolism)
ANAEROBIC DEHYDROGENASES

Anaerobic Dehydrogenases

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catalyzes to remove

hydrogen from substrates.

With the help of coenzymes

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NAD+/NADP+/FAD.

DEHYDROGENASES CANNOT

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USE OXYGEN AS A HYDROGEN

ACCEPTOR

MXH2 Anaerobic Dehydrogenase MX

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NAD+ NADH+ H+ (Non auto oxidizable)

Enter Electron Transport Chain

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For its reoxidation
Coenzymes temporarily accept the

hydrogen from substrates and get

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reduced to

NADH+ H+

FADH2

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NADPH+H+

FMNH2

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Reduced coenzymes formed in

Anaerobic Dehydrogenase

reactions are :

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Non autoxidizable/not reoxidized

at reaction level.
Reduced coenzymes

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NADH+H+ and FADH2

formed at Anaerobic

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Dehydrogenase reaction

Has to enter ETC for its

reoxidation.

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Oxygen is involved

indirectly at an end of

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ETC as electron and

proton acceptor .

Metabolic water is an

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end product of ETC.
Remember

Reduced coenzyme NADPH+H+

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do not enter ETC

NADPH+H+ is utilized as

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reducing equivalent for

reduction reactions catalyzed

by Reductases.

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NAD+ Dependent Anaerobic Dehydrogenases

Enzymes

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Pathway /Reaction

Glyceraldehyde -3-PO4

Glycolysis

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Dehydrogenase

Pyruvate Dehydrogenase

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PDH Complex

Isocitrate Dehydrogenase

TCA cycle

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Ketoglutarate Dehydrogenase

TCA cycle

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Malate Dehydrogenase

TCA cycle

Lactate Dehydrogenase

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Pyruvate/Lactate metabolism

Glutamate Dehydrogenase

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Glutamate metabolism

Hydroxy Acyl Dehydrogenase

Beta Oxidation of Fatty acids

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NADP+ Dependent

Dehydrogenases

Glucose -6-Phosphate Dehydrogenase

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( HMP Shunt)

Phospho Gluconate Dehydrogenase

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(HMP Shunt)

Note NADPH+H+does not enter ETC for

its reoxidation instead they are involved in

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reduction reactions.

FAD Dependent Anaerobic

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Dehydrogenases

Succinate Dehydrogenase
(TCA Cycle)
Acyl CoA Dehydrogenase

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( Oxidation Of Fatty Acids)
FMN Dependent Anaerobic

Dehydrogenase

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NADH Dehydrogenase

(Warburg's Yellow

Enzyme)

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First Component of ETC/

Complex I of ETC

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OXYGENASES

Oxygenases add Oxygen

atom from molecular oxygen

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(O2) into substrate.

Form Oxidized Products
OXYGENASES CATALYZE

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DIRECT TRANSFER AND

INCORPORATION OF OXYGEN

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INTO A SUBSTRATE MOLECULE

Mono Oxygenases

Mono Oxygenases add one oxygen

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atom from molecular oxygen to the

substrate.

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Forms Hydroxyl group (-OH )
Monoxygenases are also termed as

Hydroxylases or Mixed Function

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Oxidase.
AH + O2+BH2 Mono Oxygenase AOH+ B+H2O

Tyrosine+O2+THBP Tyrosine

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DOPA+DHBP+H2O

Hydroxylase

Examples Of Mono Oxygenases

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Phenylalanine Hydroxylase
(Phenylalanine to Tyrosine)
Tryptophan Hydroxylase
(Tryptophan to 5HydroxyTryptophan)

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25 Hydroxylase
(Vitamin D - Cholecalciferol activation)
1 Hydroxylase
(Vitamin D - Cholecalciferol activation)
Di Oxygenases

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Dioxygenases are true Oxygenases
Incorporates two Oxygen atoms

from O2.

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A+ O2 Dioxygenase AO2

Examples Of Dioxygenases

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Tryptophan Di Oxygenase/

Tryptophan Pyrrolase

(Tryptophan NFormyl Kynurenine )

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PHPP Dioxygenase
Cysteine Dioxygenase
Homogentisate Oxidase

(Homogentisate to 4 Maleyl Acetoacetate)

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Cytochromes P450

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Are Monooxygenases

Important in Steroid Metabolism

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& for

Detoxification of Many Drugs
Oxidases

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Oxidases involve activated

molecular Oxygen as

Hydrogen (electron and proton )

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acceptor.

Oxidases Reduce Oxygen to

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form Water (H2O)

OXIDASES USE OXYGEN

AS A HYDROGEN ACCEPTOR

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AH2 + ? O2 Oxidase A+ H2O

Tyrosine+ O2 Tyrosinase -Cu++ DOPA +

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H2O
Examples Of Oxidases

Cytochrome Oxidase-Classic Example

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(Hemoprotein ETC enzyme)

Ascorbate Oxidase
Mono Amine Oxidase
Catechol Oxidase

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HYDROPEROXIDASES USE HYDROGEN PEROXIDE

OR AN ORGANIC PEROXIDE AS SUBSTRATE

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Hydroperoxidases detoxify

Hydrogen Peroxide in body.

H2O2 is a substrate/reactant

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for Hydroperoxidases.
Hydroperoxidases are

Hemoproteins.

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Contains loosely bound

Heme as prosthetic

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group.

Hydroperoxidases prevent

accumulation of H2O2 in cells.

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H2O2 if accumulated in cells is

toxic

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Leads to disruption of

membranes(Hemolysis).

Increases risk of cancer and

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atherosclerosis.
Specific Examples Of

Hydroperoxidases

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Peroxidases
Catalase

Peroxidases Reduce Peroxides Using Various

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Electron Acceptors

Indirectly react with H2O2

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Glutathione Peroxidase
(In R.B.C's)
Leukocyte Peroxidase
(In W.B.C's)

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H2O2 + 2 GSH Glutathione Peroxidase 2H2O + GSSG
(Reduced (Oxidized

active Form)

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inactive Form)



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Catalase

Directly reacts with H2O2.
Associated with Aerobic

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Dehydrogenase catalyzed

reaction.

2H2O2 Catalase 2H2O +O2

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Biological Oxidation Process

Electron Transport Chain

(ETC)

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Oxidative Phosphorylation

Synonyms Of ETC
1. Electron Transport Chain (ETC)

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2. Oxidative Phosphorylation
3. Electron Transport System (ETS)
4. Fate of Reduced Coenzymes of

FADH2 and NADH+H+

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5. Respiratory Chain
6. Internal/Cellular Respiration
7. Tertiary metabolism
8. Final Oxidative Pathway

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What is Electron Transport Chain?
Electron Transport chain

Biological oxidation process very vital for human

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being survival

Truly Aerobic in nature(indispensable on O2)

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Located and operated at inner membrane of

Mitochondria

Alternate Oxidation and Reduction Reactions

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carried out in process

What is Oxidative Phosphorylation?

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Oxidation process (ETC) is tightly

coupled with Phosphorylation of

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ADP with pi to generate ATP.

Illustrated as Sun and Day Light

Oxidative

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Phosphorylation is a

major mode of ATP

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generation in

human body
What is Fate of ETC/

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Oxidative Phosphorylation ?

REOXIDIZES

REDUCING EQUIVALENTS

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(NADH+ H+ and FADH2)

GENERATED DURING ANAEROBIC

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DEHYDROGENASE REACTION
Electron Transport Chain

On Operation

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Transports Electrons and Protons

Through series of ETC components

Finally H2 is received by activated

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molecular Oxygen (1/2 O2)

Generates significant byproduct ATP

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and metabolic water at end of process

Condition In which ETC Operates

ETC operates in truly aerobic

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condition.

Oxygen unloaded at cellular

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level by HbO2

Gets utilized at an end of ETC

process. (Respiratory Chain)

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Site Of

Electron Transport Chain

OR

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Oxidative Phosphorylation

ETC is located and operated

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in all cells which contain

Mitochondria (Power house

of Cell)

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(Except mature Erythrocytes which are

devoid of mitochondria)

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Location of Mitochondrial ETC Complexes

? Inner membrane of Mitochondria

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? Rich In Cardiolipin

Components and Enzymes of ETC

are arranged towards inner

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surface of inner membrane of

mitochondria as:

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Vectorial conformation

Increased order of positive redox

potential

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Number of Mitochondria Vary in

Different cells

Number of Mitochondria changes

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from cell to cell , tissue to tissue,

organ to organ, organism to

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organism

Factors Responsible For Number of

Mitochondria in Cell

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Type of cell, organ and its

function

Metabolic status of an individual

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Physical activity of an individual

How much energy cell needs to

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produce?

High number of Mitochondria present in

Heart, Rod cells, Sperm,

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ciliated cells

Muscle cells for example, contain more

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number of mitochondria compared to

Kidney cells.

Marathon runners have more number of

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mitochondria in their leg muscle cells than

people with desk jobs
Components Of ETC

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Series Of Protein Complexes

Flavoproteins & Iron-Sulfur

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Proteins (Fe-S),Cytochromes

are Components

of Respiratory Chain Complexes

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1.Flavo Protein- (First Component)
NADH Dehydrogenase-FMN and FeS

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centers(Warburg's Yellow Enzyme)

2. Coenzyme Q/ Ubiquinone

3. Series of Cytochromes-

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Cytochrome b-Cytochrome c1-

Cytochrome c- Cytochrome aa3

Coenzyme Q / Ubiquinone

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Coenzyme Q (CoQ)/ Ubiquinone)

is located in lipid core of

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mitochondrial membrane.

It is a Quinone derivative
Lipophilic dissolves in

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hydrocarbon core of a membrane.
Coenzyme Q is very

hydrophobic.
Coenzyme Q has a long Poly

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isoprenoid tail, with multiple

units of isoprene.

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In human cells, most often n = 10

Q10 isoprenoid tail is longer than

width of a bilayer.

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Coenzyme Q functions as a

mobile e- carrier within

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mitochondrial inner

membrane.

Its role in trans-membrane H+

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transport coupled to e-

transfer (Q Cycle).
Coenzyme Q

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Accepts

Both Protons and Electrons

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Quinone ring of

coenzyme Q can

be reduced to

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Quinol in a 2e-

reaction:

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Q + 2 e- + 2 H+ QH .

2
When bound to special sites in respiratory complexes,

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CoQ can accept 1 e- to form a semiquinone radical (Q?

-).

Thus CoQ, like FMN, can mediate between 1 e- & 2 e-

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donors/acceptors.

Cytochromes

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Cytochromes are Hemoproteins

conjugated proteins in ETC

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Carrier of electrons

Contain heme as prosthetic group

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Cytochrome Heme

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Cytochrome Heme Iron is in

transitional state

Carries only electrons

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Fe (III) + e- Fe (II)
Only one electron is transferred at

a time.
Cytochrome heme iron

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can undergo 1 e-

transition between ferric

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and ferrous states:

Fe+++ + e- Fe++
(oxidized) (reduced)

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Cytochromes May Also Be

Regarded as Dehydrogenases

Series of Cytochromes b, c , c, aa

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1

3

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relay electrons (one at a time, in this

order
Cytochrome c is a small,

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water soluble protein with a

single heme group.

Cytochromes a & a3 are

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often referred to as

Cytochrome Oxidase

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/complex IV

Cytochrome aa3 has Fe and

Cu.

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All Cytochromes

except Cytochrome

Oxidase are Anaerobic

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Dehydrogenase

activity.

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Cytochromes absorb light at

characteristic wavelengths.

Absorbance changes upon

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oxidation/reduction of

Heme Iron
Components of Respiratory Chain

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are Contained in

Protein Complexes Embedded in

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Inner Mitochondrial Membrane

Five Complexes of

Oxidative Phosphorylation

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Complexes of Oxidative

Phosphorylation

There exists 5 complexes

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Processing Oxidative

Phosphorylation to generate

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ATP

Complexes are combination of one

or two components

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Complex I- NADH CoQ Reductase
NADH Dehydrogenase FMN and FeS centre
Complex II ? Succinate CoQ Reductase
Succinate Dehydrogenase FAD and FeS centre

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Complex III?CoQ Cytochrome C Reductase
Cytochrome b ? Cytochrome c1
Complex IV- Cytochrome Oxidase
Cytochrome aa3
Complex V ? ATP Synthetase

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F0 and F1 of ATP Synthase
Composition of Oxidative Phosphorylation Complexes

No. of

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Prosthetic Groups

Complex

Name

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Proteins

Complex I

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NADH ?CoQ

46

FMN,

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Reductase

9 Fe-S centers

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Complex II

Succinate-CoQ

5

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FAD, cyt b ,

560

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Reductase

3 Fe-S centrs.

Complex III

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CoQ-cyt c Reductase

11

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cyt b , cyt b , cyt c ,

H

L

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1

Fe-SRieske

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Complex IV

Cytochrome Oxidase

13

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cyt a, cyt a , Cu , Cu

3

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A

B

ETC Components Associated With

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Multiple Iron Sulfur Centers

Iron exists in Transitional State

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Responsible for Oxidation and

Reduction Reactions
Complex I,I and II contains Iron

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Sulfur Centers

Complex IV and V do not Contain

Iron Sulfur Centers

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ETC Components

With Iron Sulfur Centers

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NADH Dehydrogenase

Coenzyme Q-Cytochrome Reductase

Succinate ?Coenzyme Q Reductase

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Iron-sulfur centers (Fe-S) are prosthetic groups

containing 2, 3 , 4 or 8 iron atoms complexed to elemental

& Cysteine S.

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4-Fe centers have a tetrahedral structure, with Fe & S

atoms alternating as vertices of a cube.
Cysteine residues provide S ligands to the iron, while also

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holding these prosthetic groups in place within the protein.

Electron transfer proteins

may contain multiple Fe-S

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centers.
Iron-sulfur centers

transfer only one

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electron, even if they

contain two or more iron

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atoms, because of close

proximity of iron atoms.

Fe+++ (oxidized) + 1 e- Fe++ (reduced)

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COMPLEX IV

Cytochrome a-a3/ Cytochrome Oxidase

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large protein
Both a and a3 contain heme and Cu
Does not contain Fe ?S clusters
a3 Cu binds to oxygen and donates

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electrons to oxygen

Cytochrome a3 - only component of
ETC that can interact with O2

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Cytochrome Oxidase (complex IV) carries out

following irreversible reaction:
O + 4

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O

2

H+ + 4 e- 2 H2

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Four electrons are transferred into complex one at

a time from Cytochrome c.
Complex IV/Cytochrome

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Oxidase reduces molecular

Oxygen to water.

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Cytochrome Oxidase

Cu(II) Cu(I)

e- from cyt c to a

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Heme A and Cu act together to
transfer electrons to oxygen
Metal centers of cytochrome oxidase (complex IV):

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heme a & heme a3,

CuA (2 adjacent Cu atoms) & CuB.
O2 reacts at a binuclear center consisting of heme

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a and Cu .

3

B

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An Iron-Copper Center

in Cytochrome Oxidase

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Catalyzes Efficient O2

Reduction


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Complex V

ATP Synthase

Two units, Fo and F1

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("knob-and-stalk"; "bal on a stick")

F1 contains the catalytic subunits

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where ADP and Pi are brought

together for combination.

F0 spans the membrane and serves as

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a proton channel.

? F1 contains 5 types of

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polypeptide chains - a3b3gde

? Fo - a1b2c10-14

(c subunits form cylindrical,

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membrane-bound base)

? Fo and F1 are connected by a

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ge stalk and by exterior

column (a1b2 and d)

? Proton channel is ? between c

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ring and a subunit.


Complex V ATP Synthase

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How do ATPase and ATP Synthase

Differ?

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ATPase is an enzyme that

hydrolyze ATP to form ADP

ATP synthase synthesize ATP

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Both enzyme found in mitochondria


F- ATPase/ATP Synthase

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F-ATPase belong to

superfamily of related ATP

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Synthases

F-ATPase is a Rotating Motor
Mechanism Of Oxidative

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Phosphorylation

Salient Features/Required Criteria's

Of

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ETC/Oxidative Phosphorylation

OR

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Criteria's Required For Oxidative

Phosphorylation
1

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Arrangement Of

Electron Transport Chain

Components In Increased Order

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Of Positive Redox Potential

Redox Potentials & Redox Couples
FREE ENERGY

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CHANGES CAN BE

EXPRESSED IN TERMS

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OF REDOX POTENTIAL

Redox Potential is a

measure of tendency of a

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redox couple to accept or

donate electrons under

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standard condition.
Components that have most negative

redox potentials

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Have weakest affinity for electrons

Hence has capacity to donate its

electrons.

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Redox couple with most positive

redox potentials have

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Strongest affinity for electrons

therefore

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Possess strongest tendency to

accept electrons.
During E.T.C there is transfer

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of reducing equivalents

From low redox potential

to high redox potential.

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This exhibit free energy

change there by liberating

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heat energy

Electrons move spontaneously from

one component of ETC to another

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with a

low redox potential (a low affinity

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for electrons) to a component with a

high redox potential (a high

affinity for electrons)

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In ETC electrons move from a

carrier with

Low redox potential

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(high tendency to donate

electrons) toward carriers

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Higher redox potential

(high tendency to accept

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electrons)

Redox Couple

Components of ETC has capacity

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to exist in oxidant and reduced

forms.

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This pair is known as redox

couple

CoQ/CoQH2

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Cyt b Fe+++/Cyt b Fe++


Sequence of

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Respiratory

Electron

Carriers

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Inhibitors

in green

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2

Development Of

Proton Gradient And Proton Motive Force

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In Intermembrane Space


Complex I,I I and IV

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Pumps Protons From Matrix side

to Intermembrane Space

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and generates

Proton Motive Force


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Complex I,I I and IV

Serve as Proton Channels

Complex I ,III and IV act as a

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Proton Pump.

Pump out protons from matrix

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side to inter membrane space of

mitochondria.

Develop a proton gradient in

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inter membrane space.

This supports the mechanism of

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Oxidative Phosphorylation.


A Large Drop in Redox

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Potential across each of the

three Respiratory Enzyme

Complexes (I,III,IV).

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Provides the Energy for H+

Pumping

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3

Free Energy Change Occurs

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Due To Transport OF Proton

Pumping and Electron Exchange

During Oxidative Phosphorylation

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4

Heat Energy Generated At Certain

Specific Sites During Oxidation

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Is Transformed By Chemical

Phosphorylation Reaction of ADP

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and pi to form ATP

5

ATP Synthase /Complex V

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Activation for Phosphorylation

Reaction
Proton gradient runs

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downhill through ATP

Synthase to drive

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synthesis of ATP
qF F of ATP Synthase catalyzes

1 o

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phosphorylation reaction for ATP

synthesis

q Transport of H+ from intermembrane

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space to into the mitochondrial matrix

through ATP Synthase is mandatory.

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qTransport of at least 3 H+ per ATP is

required through ATP Synthase for its

activation and catalysis.

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Thus heat energy is

transformed to

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chemical form of

energy (ATP) in

Oxidative

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Phosphorylation.
6

Oxygen is Terminal Acceptor of

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Protons and electrons During

Oxidative Phosphorylation To

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Generate Metabolic Water

Oxygen has highest (most

positive) standard redox

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potential

Most likely to accept electrons

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from other carriers.
Electrons ultimately reduce Oxygen

to water (metabolic water)

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2 H+ + 2 e- + ? O2 -- H2O

At end of E.T.C by catalytic activity of

Cytochrome Oxidase

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Protons released at Coenzyme Q and

electrons transported by Cytochromes

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are

Accepted by activated molecular

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oxygen (1/2 O2) to form metabolic

water.


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Cytochrome oxidase

controls rate of O2

uptake which

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Means this enzyme

determines how

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rapidly we breathe.

Respired Oxygen

transported by Hb

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unloaded at tissue/

cellular level is

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utilized during E.T.C.
7

Coenzyme Q Accepts Electrons

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Via Complexes I & II
Point To Note

In ETC electrons flow from
Most electro negative potential

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NADH+H+ (-0.32) to most

electro positive potential

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(+0.82) ? O2 .

HOW

ETC /Oxidative

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Phosphorylation Operates ?


Most Oxidative

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Metabolic Pathways

(TCA and Beta Oxidation Of Fatty acids)

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Located In Mitochondrial Matrix

Generate Reduced Coenzymes
Reduced coenzymes

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NADH+H+/FADH2

Generated during Anaerobic

Dehydrogenase reactions of

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Carbohydrates, Lipids metabolic

pathways.

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Get reoxidized on entering E.T.C

Reduced coenzymes NADH+H+ and

FADH2 are formed in Mitochondrial

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matrix:

Oxidative Decarboxylation of

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Pyruvate to Acetyl CoA by PDH

complex.

Oxidation of Acetyl CoA by TCA cycle

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Beta Oxidation of fatty acids
NADH+H+ and FADH2 are

energy rich molecules

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Contains a pair of

electrons having a high

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transfer potential.

Entry Of NADH+H+ in ETC

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When NADH+H+ enters ETC reducing

equivalents Protons and Electrons are

taken up by first component

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/Complex I (Flavoproteins)

Then from complex I the reducing

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equivalents are transferred to CoQ.
Entry Of FADH2 in ETC

FADH2 is generated at Succinate

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Dehydrogenase reaction(Complex II)

FADH2 enters ETC process and its

reducing equivalents are taken up by

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CoQ .

CoQH2 then here onwards transfers

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only electrons to series of arranged

Cytochromes and Protons are

released in matrix.

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ELECTRON TRANSPORT CHAIN

Series of enzyme complexes (electron carriers)

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embedded in the inner mitochondrial membrane, which

oxidize NADH+H+ and FADH2 and transport electrons to

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oxygen is cal ed Respiratory Electron-Transport Chain

(ETC).

Sequence of Electron Carriers in ETC

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NADH FMN-Fe-S Co-Q Fe cyt b

cyt c1 cyt c cyt a cyt a3 O2

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Succinate FAD Fe

-S


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Electrons of NADH or FADH2 are used to

reduce molecular oxygen to water.

A large amount of free energy is liberated.

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The electrons from NADH+H+ and FADH2 are not

transported directly to O2 but are transferred

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through series of electron carriers that undergo

reversible reduction and oxidation.

ETC Process

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A PROTON GRADIENT POWERS THE

SYNTHESIS OF ATP

Transport of electrons from NADH or FADH2 to O2 via the

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electron-transport chain is exergonic process:
NADH + ?O2 + H+ H2O + NAD+
FADH2 + ?O2 H2O + FAD+
Go' = -52.6 kcal/mol for NADH

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-36.3 kcal/mol for FADH2

This process is coupled to the synthesis of ATP (endergonic

process)

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ADP + Pi ATP + H2O Go'=+7.3 kcal/mol
In E.T.C both Protons and Electrons

are transferred up to Coenzyme Q

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level.

At coenzyme Q level protons (2H+)are

released in the medium.

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From Coenzyme Q onwards only

electrons are transferred through a

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series of Cytochromes in E.T.C.

Electrons get transfer through

series of Cytochromes

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Cytochrome Fe is in transitional

state (Ferric/Ferrous ).
In E.T.C there are

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alternate reduction

and oxidation

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reactions.

Flow of electrons through ETC

complexes leads to pumping of

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protons out of the mitochondrial

matrix in intermembrane space.

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This accumulation of protons

generates a pH/Proton gradient

and a transmembrane electrical

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potential that creates a proton

motive force.
A Large Drop in Redox

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Potential across each of the

three Respiratory Enzyme

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Complexes (I,III,IV).

Provides the Energy for H+

Pumping

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Electron Transport (Oxidative

Process) is coupled to Phosphorylation
ATP is synthesized when 3

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protons flow back from

intermembrane space of

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mitochondria to

mitochondrial matrix through

an enzyme complex ATP

--- Content provided by‌ FirstRanker.com ---


synthase.

Oxidation of fuels and

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phosphorylation of ADP

are coupled by a proton

gradient across an inner

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mitochondrial membrane.
Thus Oxidative phosphorylation

is process in which ATP is

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formed

As a result of transfer of

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electrons from NADH or

FADH2 to O2 by a series of

electron carriers.

--- Content provided by‍ FirstRanker.com ---


Mechanism

Of

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Oxidative Phosphorylation
Oxidative Phosphorylation

Oxidation tightly coupled

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with Phosphorylation

E.T.C (Oxidation)Process

coupled with phosphorylation

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of ADP+pi to generate ATP.

Hypothesis And Theories Mechanism

--- Content provided by‌ FirstRanker.com ---

Of

Oxidative Phosphorylation

Chemical Coupling Hypothesis

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Conformational Coupling Hypothesis

Chemiosmotic Theory
Chemical Coupling Hypothesis:

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Put forward by Edward Slater (1953)

Proposed series of high energy

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phosphorylated intermediates are

produced during E.T.C operation.

Which are used to produce ATP.

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Conformational Coupling

Hypothesis

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Paul Boyer 1964
Mitochondrial Cristae undergo

conformational change in the

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components of E.T.C.

E.T.C components attain high

energy state which are responsible

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for the ATP production.


Chemiosmotic Theory

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Put forward by Peter Mitchell (1961)

(Nobel Prize, 1978)

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E.T.C process and ATP synthesis is

coupled by a proton gradient

developed in intermembrane space of

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mitochondria.

Mitchell's Postulates for

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Chemiosmotic Theory

Intact inner mitochondrial

membrane is required

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Electrons are pumped through ETC

complexes I,III and IV.

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Generates a Proton gradient and

in intermembrane space of

mitochondria.

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Proton pumps are Complexes I,

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III and IV.

Protons return through ATP synthase

Oxidative Phosphorylation

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Proton gradient in inter

membrane space creates Proton

Motive Force due to:

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Proton gradient have a

thermodynamic tendency

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Proton gradient creates

Electrochemical potential

difference

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Proton Motive Force drives the

Protons from mitochondrial

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intermembrane space back to

matrix side

Through a specific site of F0 and

--- Content provided by​ FirstRanker.com ---


F1 particle of ATP Synthase.
ATP Synthase catalyzes the

phosphorylation of ADP with pi

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In a reaction driven by movement

of H+ across the inner membrane

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back into the matrix through it.

Translocation of protons through

ATP Synthase

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Stimulates and activates ATP

Synthase

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For catalytic action of

phosphorylation- ADP with pi to

form ATP.

--- Content provided by⁠ FirstRanker.com ---


Supports mechanism of Oxidative

Phosphorylation.

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Flow of three H+ through an ATP

Synthase complex

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Brings a conformational change n

domains of ATP Synthase

Which causes the ATP synthase

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activate and catalyze

phosphorylation reaction

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To synthesize ATP from ADP + Pi.

ATP Synthase, a Molecular Mill.


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ATP synthesis at F1 results from

repetitive conformational changes
as rotates

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rotates 1/3 turn-
energy for ATP release

This process of producing ATP

--- Content provided by‍ FirstRanker.com ---


is known as oxidative

phosphorylation.

--- Content provided by‍ FirstRanker.com ---


Entire process of using Proton

gradient and proton motive

--- Content provided by‌ FirstRanker.com ---

force to make ATP is called

Chemiosmosis.
During oxidative

--- Content provided by‌ FirstRanker.com ---

phosphorylation total energy

change is released in small

increments.

--- Content provided by‌ FirstRanker.com ---


So that energy can be trapped as

chemical bond energy and form

--- Content provided by‌ FirstRanker.com ---

ATP.

Coupling of ATP synthesis to

respiration is indirect, via a H+

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electrochemical gradient.


Overview of Oxidative Phosphorylation

--- Content provided by‍ FirstRanker.com ---


+

+

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+

+ +

+

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-

-

--- Content provided by‌ FirstRanker.com ---

-

-

As electrons flow through complexes of ETC, protons are translocated

--- Content provided by‌ FirstRanker.com ---


from matrix into the intermembrane space.
The free energy stored in the proton concentration gradient is tapped

as protons reenter the matrix via ATP synthase.

--- Content provided by‌ FirstRanker.com ---

As result ATP is formed from ADP and Pi.



ATP Translocation From

--- Content provided by FirstRanker.com ---


Mitochondria

Through ATP/ADP Translocases

--- Content provided by​ FirstRanker.com ---


ATP molecules produced in Oxidative

Phosphorylation mechanism are

--- Content provided by FirstRanker.com ---

Transported out of mitochondrial

matrix through specific transporters

Operation Of ETC

--- Content provided by‌ FirstRanker.com ---




Glycolysis, Fatty

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acid oxidation

TCA cycle

supplies NADH

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and FADH2 to

the Electron

--- Content provided by‌ FirstRanker.com ---

Fatty Acids

Acetyl Co A

Transport Chain

--- Content provided by‍ FirstRanker.com ---


Pyruvate

Amino Acids

--- Content provided by FirstRanker.com ---

Glucose
WHY ETC OPERATES ?

During E.T.C operation total

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energy change is released in

small increments

So that energy can be trapped

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as chemical bond energy to

form ATP.
When two redox couples of ETC

--- Content provided by‌ FirstRanker.com ---


differ from each other by 0.22 volts

in standard redox potential.

--- Content provided by FirstRanker.com ---

At this site free energy in the form of

heat released which is more than 7.3

Kcal.

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This free heat energy is conserved to

undergo Phosphorylation reaction

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and generate chemical form of

energy-ATP.

Sites in E.T.C at which

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energy liberated is less

than 7.3 Kcal is simply

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dissipiated in the

form of heat.
Three sites in E.T.C (Complex

--- Content provided by‍ FirstRanker.com ---

I,III and IV) where heat energy

liberated more than 7.3 Kcal

Utilized for phosphorylation

--- Content provided by​ FirstRanker.com ---


reaction of ADP with pi to

form ATP.

--- Content provided by‍ FirstRanker.com ---

Electrons are transferred from

NADH+H+ O via multisubunit

2

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inner membrane complexes I, III

& IV, plus CoQ & Cytochrome c.

--- Content provided by FirstRanker.com ---

Within each complex, electrons

pass sequentially through a series

of electron carriers.

--- Content provided by⁠ FirstRanker.com ---

Complex I catalyzes

oxidation of NADH+H+ with

reduction of coenzyme Q:

--- Content provided by‍ FirstRanker.com ---


NADH + H+ + FP NAD+ + FPH2
Coenzyme Q accepts 2 e- and picks

up 2 H+ from FPH2 to yield the fully

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reduced QH2.
Succinate Dehydrogenase of the Krebs Cycle is

also called complex II or Succinate-CoQ

--- Content provided by‍ FirstRanker.com ---


Reductase.

FAD is initial e- acceptor.

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FAD is reduced to FADH2 during oxidation of

Succinate to Fumarate.

FADH2 generated by Succinate

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Dehydrogenase reaction gets reoxidized

by transfer of electrons through a series

--- Content provided by‍ FirstRanker.com ---

of 3 iron-sulfur centers to CoQ, yielding

CoQH2.

QH2 product may be reoxidized via

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complex III.

Providing a pathway for transfer of

--- Content provided by FirstRanker.com ---

electrons from Succinate into respiratory

chain.
Complex III/ Cytochrome b-c1

--- Content provided by​ FirstRanker.com ---

complex accepts electrons from

coenzyme QH2 that is generated

by electron transfer in complexes

--- Content provided by⁠ FirstRanker.com ---


I & II.

Cytochrome c resides in

--- Content provided by‌ FirstRanker.com ---

intermembrane space.

It alternately binds to

complex III or IV during e-

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transfer.
Significance Of ETC

Reduced coenzymes gets reoxidized

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to NAD+ /FAD in ETC for its

reutilization in metabolic oxidation

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reactions.

Reduced coenzymes NADH+

H+/FADH2 give its reducing

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equivalents to E.T.C components and

get reoxidized.

--- Content provided by FirstRanker.com ---

E.T.C generates chemical form of

energy ATP as a valuable by product.
P/O Ratio

--- Content provided by FirstRanker.com ---

Ratio of ATPs formed per

Oxygen reduced

OR

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Number of ATPs generated

per Oxygen atom used in

--- Content provided by FirstRanker.com ---

ETC process.
To make 1 ATP need 30 kJ/mole

There needs more than one

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proton to translocate during

ETC process to generate 1 ATP.

Ten protons are pumped out of

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the matrix during the two electrons

flowing from NADH+H+ to O2

--- Content provided by‌ FirstRanker.com ---

(Complex I, III and IV).

Six protons are pumped out of the

matrix during the two electrons

--- Content provided by​ FirstRanker.com ---


flowing from FADH2 to O2

(Complex III and IV).
Spontaneous electron flow

--- Content provided by‌ FirstRanker.com ---


through each of complexes I, III, &

IV is coupled to H+ ejection from

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matrix to intermembrane Space

A total of 10 H+ are ejected from the mitochondrial matrix per 2 e

- transferred from NADH to oxygen via the respiratory chain.

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A total of 6 H+ are ejected from the mitochondrial matrix per 2 e-

transferred from FADH2 to oxygen via the respiratory chain.
Complex I and Complex III transports 4H+

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out of the mitochondrial matrix per 2e-

transferred from NADH.

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Thus there are 2H+ per 2e- that are

effectively transported by complex

IV.

--- Content provided by FirstRanker.com ---



ATP Yield

3

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4

4

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2

10 protons translocates

per NADH+H+

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6 protons translocates

per FADH2
Proton gradient and Proton

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motive Force created as

electrons transferred to

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Oxygen forming water

10 H+ / NADH+H+
6 H+ / FADH2

--- Content provided by​ FirstRanker.com ---

F F couples ATP synthesis to H+

1 o

transport into the mitochondrial matrix.

--- Content provided by‍ FirstRanker.com ---

Transport of least 3 H+ per ATP is

required.
Translocation of 3H+

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required by ATP Synthase for

each ATP produced

1 H+ needed for transport of Pi.

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Net: 4 H+ transported for

each ATP synthesized

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through ATP Synthase.

P:O Ratio for NADH+H+

10 H+ X 1 ATP = 2.5 ATP

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4 H+

P.O Ratio for FADH2

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6 H+ X 1 ATP = 1.5 ATP

4 H+
P:O ratio for NADH: 10 H+/4H+ =

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2.5 ATP

P:O ratio for FADH2: 6 H+/ 4H+ =

1.5 ATP

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ATP Is A Valuable Byproduct Of

Oxidative Phosphorylation
ATP is a high energy phosphate

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compound

Biologically important free

--- Content provided by​ FirstRanker.com ---

nucleotide

ATP has Two High Energy Phosphate

Anhydride Bonds

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ATP is energy currency of

cell.

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Predominantly generated

through Oxidative

Phosphorylation.

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Sites Of ATP Production In ETC

3 sites Of ATP Generation in ETC

Site I/Complex I-

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Electrons transferred from Complex I to

CoQ

--- Content provided by⁠ FirstRanker.com ---

Site II/Complex III-

Electrons transferred from Cyt b to Cyt c1.

Site III/Complex IV-

--- Content provided by FirstRanker.com ---


Electrons transferred from Cytochrome

aa3/Complex IV/Cytochrome Oxidase to ?

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O2
Thus ATP Generation

Is Due To Transformation Of

--- Content provided by​ FirstRanker.com ---

Heat Energy Into Chemical Form Of Bond Energy

Which Satisfy Law Of Thermodynamics

Energy Is never Destructed

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Energy Is Transformed From One Form To Another

From One System To Another

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One Body To Another

Significance OF ATP

ATP allows coupling of

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thermodynamically

unfavorable reactions

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to favorable reactions.
Uses of ATP generated in

Oxidative Phosphorylation

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?Synthetic/Anabolic reactions
?Active transport mechanism.
?Muscular contraction
?Nerve impulse conduction.

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ATP is continually being

hydrolyzed and

regenerated

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A person at rest

consumes and regenerate

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3 ATP/ sec
Staying Alive Energy Wise

We need 2000 Cal/day or 8,360 kJ of energy per day
Each ATP gives 30.5 kJ/mole of energy on hydrolysis

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We need 246 moles of ATP
Body has less than 0.1 moles of ATP at any one time
We need to make 245.9 moles of ATP
Each mole of Glucose yields 38 ATPs or 1160 kJ
We need 7.2 moles of Glucose (1.3 kg or 2.86 pounds)

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Each mole of Stearic acid yields 147 ATPs or 4,484 kJ
We need 1.86 moles of stearic acid (0.48 kg or 1.0

pound of fat)

--- Content provided by FirstRanker.com ---

Remember

CoQ accepts electrons and

Protons by complexes I and II

--- Content provided by‌ FirstRanker.com ---


Acceptance of Protons and

Electrons from Complex II by

--- Content provided by FirstRanker.com ---

CoQ does not generate ATP
E.T.C is a Mode For Free Radical

Generation

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During E.T.C operation there

occurs leakage of small amounts

of electrons

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Which are transferred directly

to oxygen to form super oxide

--- Content provided by FirstRanker.com ---

ion (Free radicals/ROS)


What is a Free Radical ?

--- Content provided by​ FirstRanker.com ---

Any chemical species with one of more

unpaired electrons

Unstable/Highly Reactive to get

--- Content provided by​ FirstRanker.com ---


stabilized

Powerful Oxidant
Short half life (nanoseconds)

--- Content provided by⁠ FirstRanker.com ---

Can exist freely in environment

Main Factors Responsible for

ETC

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Factors For Universal Metabolism

Nutrition
Environment
Life Style Habits

--- Content provided by‍ FirstRanker.com ---


Factors Associated To ETC

Metabolites- Carbs ,Proteins , Lipids

--- Content provided by‌ FirstRanker.com ---

Vitamins , Minerals and Antioxidants

Oxygen Concentration

Respiration Process

--- Content provided by‍ FirstRanker.com ---


Hemoglobin Structure and Function

Mitochondrial DNA

--- Content provided by‌ FirstRanker.com ---

Metabolic Status

Oxidative Stress
REGULATORS OF OXIDATIVE

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PHOSPHORYLATION

Important Direct Substrates

Regulators Of Oxidative Phosphorylation

--- Content provided by‍ FirstRanker.com ---


and ATP Generation

NADH/FADH2

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O2

ADP and pi
Indirect Substances Involved

--- Content provided by‌ FirstRanker.com ---

Glucose
Fatty acids
Insulin
Amino acids and Proteins
Iron

--- Content provided by‌ FirstRanker.com ---

Vitamin C
Vitamin B Complex members- Niacin,

Riboflavin

--- Content provided by‍ FirstRanker.com ---

ATP/ADP Ratio

Regulates Mechanism Of

Oxidative Phosphorylation

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Respiratory Control

The most important factor in determining the rate of

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oxidative phosphorylation is the level of ADP.
The regulation of the rate of oxidative phosphorylation by

the ADP level is cal ed respiratory control

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ADP and pi is required for ETC

process.

--- Content provided by‍ FirstRanker.com ---

Intramitochondrial ratio

ATP/ADP is a control

mechanism

--- Content provided by​ FirstRanker.com ---

At high ATP/ADP ratio
ATP acts as an allosteric

inhibitor for Complex IV

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(Cytochrome Oxidase)

Inhibition is reversed by

increasing ADP levels.

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ADP levels reflect rate of ATP

consumption and energy state

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of the cell.

At low ADP levels ? Low

oxidative phosphorylation

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Electron transport is tightly coupled to

phosphorylation.

ATP cannot be synthesized by

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oxidative phosphorylation unless there

is heat energy released from electron

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transport.

Electrons do not flow through the

electron-transport chain to O2 unless

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ADP is phosphorylated to ATP.

Inhibitors OF

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ETC Complexes

OR

Inhibitors Of

--- Content provided by FirstRanker.com ---


Oxidative Phosphorylation
ETC Complexes Inhibitors

OR

--- Content provided by‌ FirstRanker.com ---


Interruptors of Oxidative Phosphorylation

Mechanism

--- Content provided by⁠ FirstRanker.com ---

Enemies/Distractors of ETC components

who stop its normal operation.

Block ETC operation and stop ATP

--- Content provided by‍ FirstRanker.com ---


generation.

ETC Complexes Inhibitors

--- Content provided by​ FirstRanker.com ---

Chemical compounds having affinity for ETC

components/complexes

Chemically interact with ETC complexes, bind and

--- Content provided by​ FirstRanker.com ---


inactivate them

Affects normal functional operation of ETC

--- Content provided by⁠ FirstRanker.com ---

Low/No ATP production

Cessation of cellular activities
Complex I/Site I - E.T.C Inhibitors

--- Content provided by‍ FirstRanker.com ---

vAmobarbital /Amytal
vRotenone (Fish/Rat Poison)
vMercurials
vPiercidin ?A

--- Content provided by‍ FirstRanker.com ---


(Volatile Anesthetics)
vHalothane (Malignant Hyperthermia)
vFluothane
vIsoflurane

--- Content provided by​ FirstRanker.com ---

vSevoflurane

Complex III/ Site II -E.T.C Inhibitors

vBritish Anti Lewisite ( BAL)

--- Content provided by​ FirstRanker.com ---

vAntimycin ?A
vDimercaprol


Complex IV/Site III /Cytochrome

--- Content provided by⁠ FirstRanker.com ---


Oxidase Inhibitors :



--- Content provided by​ FirstRanker.com ---

vCyanide
vCarbon Monoxide
vH2S
vAzide
Complex V Inhibitors

--- Content provided by‍ FirstRanker.com ---


ATP Synthase Inhibitors

Oligomycin

--- Content provided by‍ FirstRanker.com ---

Fo particle of ATP Synthase serve as

proton channel

An antibiotic Oligomycin binds with

--- Content provided by FirstRanker.com ---


Fo particle of ATP Synthase

Do not translocate Protons through

--- Content provided by​ FirstRanker.com ---

it.

Inhibits activation of ATP Synthase

phosphorylation of ADP to ATP.

--- Content provided by‌ FirstRanker.com ---

Atractyloside

A Glycoside prevents

translocation of ADP across

--- Content provided by⁠ FirstRanker.com ---


mitochondrial membrane.

Make it unavailable for

--- Content provided by FirstRanker.com ---

phosphorylation reaction

Bongregate

Pseudomonas toxin has

--- Content provided by‌ FirstRanker.com ---


inhibitory action similar

like Atractyloside.
Artificial Electron Acceptors/

--- Content provided by FirstRanker.com ---


Distractors Of ETC

These chemicals

--- Content provided by FirstRanker.com ---

arrest respiration

by inhibition of

ETC complexes

--- Content provided by⁠ FirstRanker.com ---



Specific inhibitors of

Electron Transport Chain

--- Content provided by⁠ FirstRanker.com ---


and

ATP-Synthase

--- Content provided by​ FirstRanker.com ---



Uncouplers Of

Oxidative

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Phosphorylation

What are Uncouplers?

--- Content provided by⁠ FirstRanker.com ---


Uncouplers are chemical agents

Uncouplers are mostly lipid soluble

--- Content provided by‍ FirstRanker.com ---

aromatic weak acids

They Uncouple/Delink two tightly

coupled natural processes

--- Content provided by⁠ FirstRanker.com ---


E.T.C (Oxidation) uncoupled from

Phosphorylation (ATP generation)

--- Content provided by‍ FirstRanker.com ---

They just carry out Oxidation

without Phosphorylation


--- Content provided by FirstRanker.com ---

Uncouplers break the

connection between

Electron Transport Chain and

--- Content provided by‍ FirstRanker.com ---


Phosphorylation

Electron transport is a motor

--- Content provided by⁠ FirstRanker.com ---

Phosphorylation is the transmission

Uncouplers put the car in NEUTRAL

Uncouplers Action Il ustrates

--- Content provided by‌ FirstRanker.com ---


As Total Solar Eclipse
Uncouplers just bring oxidation

(E.T.C/Sun Rise) without

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phosphorylation(Interrupted Sun Light)

Uncoupler (Moon In between) inhibits

--- Content provided by⁠ FirstRanker.com ---

generation of ATP ( Dark/No Day)

Types Of Uncouplers
Physiological Uncouplers

--- Content provided by⁠ FirstRanker.com ---

Thermogenin /Uncoupling Protein-1
Excess of Thyroxine
Long Chain Fatty acids
Unconjugated Hyperbilirubinemia

--- Content provided by FirstRanker.com ---

Chemical Uncouplers

2,4 Di Nitro Phenol
Di Nitro Cresol
Dicumarol

--- Content provided by FirstRanker.com ---

Aspirin
p-Triflouromethoxy Carbonyl Cyanide

Phenylhydrazone (FCCP)

--- Content provided by‍ FirstRanker.com ---

Valinomycin
Pentachlorophenol
Snake Venom-Phospholipases


--- Content provided by‍ FirstRanker.com ---

Mode Of Action Of Uncouplers

Certain Uncouplers are ionophores,

lipophilic substances.

--- Content provided by‌ FirstRanker.com ---


They carry protons from

intermembrane space across

--- Content provided by⁠ FirstRanker.com ---

mitochondrial membrane to matrix

From site other than specific site.

(i.e not through F0 and F1 particles of

--- Content provided by​ FirstRanker.com ---


ATP Synthase).
Certain Uncouplers changes

permeability of mitochondrial

--- Content provided by FirstRanker.com ---


membrane to protons.

Translocate protons easily

--- Content provided by‌ FirstRanker.com ---

through mitochondrial

membrane.


--- Content provided by FirstRanker.com ---

2,4 DNP dissolve in membrane and function as

carriers for H+.


--- Content provided by FirstRanker.com ---


Uncouplers block oxidative phosphorylation by

dissipating H+ electrochemical gradient.
Protons pumped out leak back into mitochondrial

--- Content provided by‍ FirstRanker.com ---


matrix,
preventing development of proton gradient and

proton motive force.

--- Content provided by​ FirstRanker.com ---


ATP Synthase reaction runs backward

in presence of an uncoupler.
w Hydrolysis of ATP is spontaneous.

--- Content provided by⁠ FirstRanker.com ---

Thus Uncouplers by their action

deplete proton gradient of

intermembrane space during

--- Content provided by⁠ FirstRanker.com ---


ETC operation.

Uncouplers Dissipate More Heat

--- Content provided by​ FirstRanker.com ---

Uncouplers Do not allow to develop

required proton gradient and

Do not form proton motive force in the

--- Content provided by FirstRanker.com ---


intermembrane space of mitochondria

No translocation of Protons through ATP

--- Content provided by‌ FirstRanker.com ---

Synthase

Causes no stimulation or activation of ATP

Synthase

--- Content provided by FirstRanker.com ---


No catalysis of Phosphorylation of ADP with

pi to generate ATP.
During uncoupling phenomena

--- Content provided by FirstRanker.com ---




Free energy released as Heat energy

--- Content provided by​ FirstRanker.com ---

more than 7.3 Kcal is not conserved for

Phosphorylation reaction dissipiated as

it is in form of heat

--- Content provided by​ FirstRanker.com ---


A very high heat energy released then

causes swelling of Mitochondria and

--- Content provided by FirstRanker.com ---

exhibit malignant hyperthermia.

Physiological Uncoupling

By

--- Content provided by‍ FirstRanker.com ---


Uncoupling Protein (UCP-1)



--- Content provided by FirstRanker.com ---

An Uncoupling Protein (UCP-1)/

Thermogenin is produced in brown

adipose tissue of newborn mammals and

--- Content provided by​ FirstRanker.com ---


hibernating mammals.

This UCP-1 protein of an inner

--- Content provided by‍ FirstRanker.com ---

mitochondrial membrane functions as a

H+carrier.


--- Content provided by FirstRanker.com ---


Uncoupling by UCP-1 protein blocks

development of a H+ electrochemical

--- Content provided by‌ FirstRanker.com ---

gradient, thereby stimulating

respiration.

Free energy of ETC is dissipated as heat.

--- Content provided by⁠ FirstRanker.com ---


Uncoupling of ETC and

phosphorylation occurs in animals

--- Content provided by​ FirstRanker.com ---

as a means to produce heat

Non shivering thermogenesis
Occurs in brown adipose tissues

--- Content provided by​ FirstRanker.com ---

(rich in mitochondria)


Significance Of Physiological

--- Content provided by FirstRanker.com ---

Uncouplers

In extreme cold conditions and in

hibernating animals

--- Content provided by FirstRanker.com ---


Physiological Uncouplers bring

uncoupling phenomena

--- Content provided by FirstRanker.com ---

The heat liberated inside body helps

to restore and maintain body

temperature.

--- Content provided by​ FirstRanker.com ---


Brown adipose
(fat) cells
contain natural
Uncouplers to

--- Content provided by‌ FirstRanker.com ---

warm animals
cold adaptation
and hibernation.


--- Content provided by​ FirstRanker.com ---

As per the Required condition Of

Body

This "non-shivering

--- Content provided by‌ FirstRanker.com ---


thermogenesis" is costly in

terms of respiratory energy

--- Content provided by⁠ FirstRanker.com ---

Heat energy unavailable for

ATP synthesis

But provides valuable warming

--- Content provided by FirstRanker.com ---


to an organism.

Effect Of Poor Antioxidant Activity

--- Content provided by FirstRanker.com ---


ETC Inhibitors and Uncouplers

Any compound that stops

--- Content provided by⁠ FirstRanker.com ---

electron transport will stop

respiration...this means you

stop breathing

--- Content provided by‍ FirstRanker.com ---


Electron transport can be

stopped by inhibiting ATP

--- Content provided by​ FirstRanker.com ---

synthesis

An uncoupler breaks the

connection between ATP

--- Content provided by‌ FirstRanker.com ---


synthesis and electron

transport
Shuttle Systems

--- Content provided by​ FirstRanker.com ---


Shuttling Reducing Equivalents

OF NADH+H+

--- Content provided by‌ FirstRanker.com ---

from Cytosol into the

Mitochondrion


--- Content provided by⁠ FirstRanker.com ---

Shuttle

A vehicle or aircraft that travels regularly

between two places

--- Content provided by⁠ FirstRanker.com ---


Biochemical shuttle is a biochemical

system for translocating Protons and

--- Content provided by⁠ FirstRanker.com ---

electrons produced during Glycolysis

Across a semipermeable inner membrane

of mitochondrion

--- Content provided by⁠ FirstRanker.com ---


For oxidative phosphorylation mechanism

NADH+H+ is generated in the cytosol during

--- Content provided by⁠ FirstRanker.com ---

Glycolysis
Cytosolic NADH+H+ itself is not

carried across the mitochondrial

--- Content provided by‌ FirstRanker.com ---

membrane.

Instead its Protons and

Electrons of NADH+H+ are

--- Content provided by​ FirstRanker.com ---


carried through shuttle

systems.

--- Content provided by‍ FirstRanker.com ---

Since NAD+ and NADH +H+ are

impermeable to an inner

mitochondrial membrane

--- Content provided by⁠ FirstRanker.com ---


This reducing equivalents must be

shuttled into mitochondrial matrix

--- Content provided by⁠ FirstRanker.com ---

before they can enter the ETC.
Cytosolic NADH+H+

Enter Mitochondria

--- Content provided by FirstRanker.com ---

via

2 Shuttle Systems

Two shuttles Involved:

--- Content provided by​ FirstRanker.com ---


Malate-Aspartate Shuttle

Glycerol 3-phosphate Shuttle

--- Content provided by‌ FirstRanker.com ---


Malate-Aspartate Shuttle

Malate/Aspartate Shuttle System
Malate Aspartate Shuttle

--- Content provided by‍ FirstRanker.com ---


Active in Heart and Liver.
2.5 molecules of ATP are produced

Glycerol-3-Phosphate Shuttle

--- Content provided by FirstRanker.com ---



Glycerol-3-Phosphate Shuttle

Glycerol 3 Phosphate Shuttle

--- Content provided by‌ FirstRanker.com ---

Glycerol Phosphate Shuttle

Active in Skeletal muscles and

Brain

--- Content provided by‍ FirstRanker.com ---


FADH2 formed in this enter the

electron-transport chain through

--- Content provided by⁠ FirstRanker.com ---

CoQ

Generates only 1.5 molecules of

ATP

--- Content provided by‌ FirstRanker.com ---


Summary of Shuttle Systems

Total ATPs Generated / 1 Glucose Oxidation

--- Content provided by‌ FirstRanker.com ---

Heart and Liver

32.0 ATP

Uses Malate Aspartate Shuttle

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Muscle and Brain

30.0 ATP

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Uses Glycerol phosphate Shuttle
Factors Affecting

Oxidative Phosphorylation

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Mechanism

Oxygen supply to cells
Hemoglobin structure and function
Respiratory system and its function

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Mitochondrial structure and ETC

components.
Presence of Nutrients
Enzyme function and

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Coenzymes availability

Adequate amount of ADP

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and pi.

Presence of ETC inhibitors

Pathological Conditions Affecting

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Oxidation Phosphorylation

Mechanism

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Which

Lower Down ATP Production
1. Hypoxia

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2. Anemia

3. Ischemia


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4. Hemoglobinopathies

5. Emphysema

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6. Respiratory Distress Syndrome

7. Asthma

8. Prolonged Starvation

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9. Malnutrition

10. Diabetes mellitus

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11. ETC inhibition by chemicals/drugs

12. Inherited Disorders of Mitochondria

Inherited /Genetic Disorders

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Related To Mitochondrial

Oxidative Phosphorylation

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Mechanism
Mitochondrial DNA

Mitochondrial genes encode for ETC complexes

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Complex I
Complex III
Complex IV
Complex V

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Mutations in any one or more genes of

mitochondrial DNA controlling mechanism of Oxidative

phosphorylation lead to its inherited disorders

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1. MELAS

An inherited disorder caused due to defect

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of complex I or IV of E.T.C

Associated with

Mitochondrial Myopathy

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Encephalopathy
Lactate accumulation
Acidosis
Stroke
2. Fatal Infantile Mitochondrial Myopathy

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Defect in E.T.C components

located in mitochondria

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Cytochrome c Oxidase defect
Associated with renal

dysfunction.

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Mostly fatal in early age

3. Leber's Hereditary

Optic Neuropathy (LHON)

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Caused due to mutations in

mitochondrial DNA

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Affects oxidative

phosphorylation mechanism

Loss of bilateral vision due to

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neuroretinal degeneration.


Mutant Genes Of LHON

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4. Mitochondrial DNA Deletion

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Syndrome




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5. Luft's Disease

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Luft's Disease is a mitochondrial disease

First patient who was diagnosed with this

disease was a 30 year old Swedish woman by

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Dr Rolf Luft

Caused by abnormal mitochondria
Biochemical Cause

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Mitochondria Respire Wildly

Respiratory control is lost
Partial Uncoupling is caused by an abnormality in

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mitochondrial membrane

Electron transport is only loosely coupled to ATP

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production

Oxidation process proceed independent of ADP

phosphorylation to generate ATP

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An extra energy evolves in form of heat
This elevates body temperature up to 38.4 ?C which

raises BMR

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Luft's Disease Is Characterized By

Abnormal excessive production of heat

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Characterized by hypermetabolism and

abnormal transpiration.

Patient experiences excessive sweating

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during winter

Make them to change their clothes 10 times a

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day.
Onset is in childhood

Thyroid function is normal

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Since there is less ATP production

and an extra energy is lost in the

form of heat

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Metabolic processes are stimulated

Luft's Disease

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Non Thyroidal Hypermetabolism

Due to high BMR and low ATP production
High caloric intake
There is failure to put on weight despite a good diet

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There is progressive weight loss despite increased food

intake

Excessive perspiration

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Excessive thirst indicate a state of severe hyper

metabolism of non thyroid origin (since thyroid hormones

-T3 and T4 are normal)

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Manifestations of Luft's Disease

Heat intolerance

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Profuse perspiration
Polydipsia without polyuria
Severe hyper metabolism
Polyphagia
Muscular wasting and weaken

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Absent deep reflexes, and Resting

tachycardia.

Multiorgan Dysfunction Risk In

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Luft's Disease
Case Study

An elderly couple was brought by ambulance to an emergency

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department after their daughter noticed that they were both

acting "strangely." The couple had been in good health prior

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to the weekend. Their daughter had gone out to spend the

week-end with her friends. The couple had been snowed in at

their house until the snowplows cleared the roads. They had

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plenty of food and were kept warm by a furnace and blankets.

On reaching home after two days, their daughter noticed that

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they both were complaining of bad headaches, confusion,

fatigue, and some nausea. On arrival to an emergency

department, both patients were afebrile with normal vital

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signs and O2 saturation of 99 percent on 2 L of O2 by nasal

cannula. Their lips appeared to be very red. Both patients were

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slightly confused but otherwise oriented. The physical

examinations were within normal limits.

Carboxyhemoglobin levels were drawn and were elevated.

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What is most likely cause of these patients' symptoms?


Questions

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Long Essays.

Q.1 Define Biological oxidation.

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Enumerate and Describe various

enzymes carrying out biological

oxidation reactions with suitable

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examples.
Q.2 Describe Respiratory chain and

Give its significance.

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OR
Explain the Electron. Transport chain

(E.T.C.) and its significance.

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OR
How the reduced equivalents generated

in anaerobic dehydrogenase reactions

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are reoxidized.

Q.3 What is oxidative

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phosphorylation? Explain

the mechanism with

respect to various theories

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and hypothesis.
Short Notes

Cytochromes

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Inhibitors of E.T.C
Shuttle systems and its

significance

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Inhibitors and Uncouplers of

oxidative phosphorylation

Complexes of E.T.C.

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Redox potential and free energy

changes.

Inherited Disorders related to E.T.C.

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abnormality.

ATP ? Mode of its formation and

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it's role in the Body.
Short Answer Questions

Give the sites for ATP generation

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of in E.T.C.

Enumerate the High energy

compounds of our body

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Substrate level phosphorylation

and it's importance.

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Enumerate the Enzymes

catalyzing Biological oxidation

reactions. Write the class to

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which these enzymes classified.

Inherited Disorders of

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Mitochondrial Dysfunction
Define P.O ratio. What is the P:O

ratio for reduced NADH+H+ &

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FADH2 respectively.

List the components of E.T.C. and

their location.

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Redox couple & Redox potential.

FlavoProteins
Product of Aerobic and

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Anaerobic dehydrogenation

reactions.

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Write enzymes catalyzing

Aerobic and Anaerobic

dehydrogenation reaction's

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during metabolism.
THANK YOU

Laboratory data showed lactic acidosis,

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Proteinuria
Glycosuria and
Generalized aminoaciduria
Muscle biopsy showed large clumps of

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granules positive with oxidative enzyme

stains and increased lipid droplets.

Ultrastructural studies showed large

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aggregates of mitochondria, many of which

were greatly enlarged and contained

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disoriented or concentric whorls of cristae

and paracrystalline inclusions.
A 1-month-old boy was admitted because of failure to thrive.

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He was floppy and had bilateral ptosis, diminished reflexes, and

poor suck. He had aspiration pneumonia, developed seizures,

and died at age 3 1/2 months.

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He was an only child, and family history was negative.
Cytochrome c oxidase was absent in fresh frozen sections

by histochemical staining.

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By biochemical assay, cytochrome c oxidase (cytochrome aa3)

was 6% of normal in muscle biopsy and undetectable in autopsy

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muscle; spectra and content of cytochromes showed lack of

cytochrome aa3, decreased cytochrome b and normal

cytochrome cc1.

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In kidney, cytochrome-c-oxidase activity was 38% of normal

and spectra showed decreased cytochromes aa3 and b.

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The association of fatal infantile mitochondrial myopathy,

lactic acidosis and renal dysfunction was previously reported

by Van Biervliet et al and appears to be a distinct nosologic

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entity, one of the few biochemically defined mitochondrial

myopathies.

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A case of cytochrome c oxidase deficiency primarily affecting

skeletal muscle is described. The child was admitted at 4

weeks due to failure to thrive and examination at that time

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revealed weakness and hypotonia. His condition deteriorated

until at 11 weeks respiratory arrest necessitated artificial

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ventilation and death occurred at 14 weeks. Biochemical

investigation showed lactic acidemia and generalized

aminoaciduria. Histochemical examination of muscle obtained

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at biopsy showed strong reactions for some oxidative enzymes,

but by contrast cytochrome c oxidase could not be detected.

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Cytochrome c oxidase activity was less than 5% of control

values in an extract of fresh muscle. The reduced-minus

oxidized absorption spectra of muscle mitochondrial fractions

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prepared from post-mortem tissue showed an absence of

cytochrome aa3 and a partial deficiency of cytochrome b. Ultra

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-structural examination showed abnormal mitochondria with

loss of cristae and an abnormal granular matrix. The family

history suggests autosomal recessive inheritance.

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