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? Proteins similar to importins, referred to as exportins, are
involved in the export of
proteins, tRNA molecules, ribosomal subunits and
certain mRNA molecules) from the nucleus
(NESs).
? Ran proteins are involved in this process also
karyopherins
? Another system is involved in the translocation of the
majority of mRNA molecules.
? These are exported from the nucleus to the cytoplasm as
named mRNP exporter
Peroxisomes
Transport into peroxisomes
Transport into peroxisomes
? Bind to proteins with PTS1 and dock to the
translocation channel
? Protein is released
? Peroxin is recycled
Zellweger syndrome
? Usually caused by mutations in genes encoding certain
--involved in various steps of peroxisome biogenesis
? Number of peroxisomes can vary from being almost
normal to being virtually absent in some patients
? Apparent at birth and is characterized by profound
? Biochemical findings include an accumulation of very-
long-chain fatty acids, abnormalities of the synthesis of
bile acids, and a marked reduction of plasmalogens
? Two closely related conditions are neonatal
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? Proteins similar to importins, referred to as exportins, are
involved in the export of
--- Content provided by FirstRanker.com ---
many macromolecules (variousproteins, tRNA molecules, ribosomal subunits and
certain mRNA molecules) from the nucleus
--- Content provided by FirstRanker.com ---
? Cargo molecules for export carry nuclear export signals(NESs).
? Ran proteins are involved in this process also
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? The family of importins and exportins are referred to askaryopherins
? Another system is involved in the translocation of the
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majority of mRNA molecules.
? These are exported from the nucleus to the cytoplasm as
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ribonucleoprotein (RNP) complexes attached to a proteinnamed mRNP exporter
Peroxisomes
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Transport into peroxisomes
Transport into peroxisomes
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? Peroxins - peroxisome transport receptors? Bind to proteins with PTS1 and dock to the
translocation channel
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? The complex is transported through the membrane? Protein is released
? Peroxin is recycled
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Zellweger syndrome
? Usually caused by mutations in genes encoding certain
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proteins--the PEX family of genes, also called peroxins--involved in various steps of peroxisome biogenesis
? Number of peroxisomes can vary from being almost
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normal to being virtually absent in some patients
? Apparent at birth and is characterized by profound
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neurologic impairment, victims often dying within a year? Biochemical findings include an accumulation of very-
long-chain fatty acids, abnormalities of the synthesis of
--- Content provided by FirstRanker.com ---
bile acids, and a marked reduction of plasmalogens
? Two closely related conditions are neonatal
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adrenoleukodystrophy and infantile Refsum disease.