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13 Cards in this Set

  • Front
  • Back
True or False?
Peroxisomes participate in a variety of diverse anabolic and catabolic functions.
What functions to peroxisomes participate in?
Plasmalogen synthesis
Bile acid synthesis
Cholesterol biosyntheis
Glyoxylate transamination
Pipecolic acid oxidation
β-oxidation of VLCFA
All peroxisomal proteins are encoded on ? genes, are translated on ?, and are selectively imported into pre-existing ?.
nuclear; polyribosomes; peroxisomal membranes
What mediates post-translational transport of peroxisomal proteins to the peroxisomes?
Peroxisomal targeting signals in the primary sequence.
What are the two peroxisomal targeting sequences?
PTS 1: carboxy terminal signal
PTS 2: amino terminal signal
To what do the PTSs bind?
Specific receptors for transport peroxisomal proteins to and into peroxisomes.
What do the PEX genes do?
The PEX genes encode for PTS receptors and proteins involved in peroxisomal biosynthesis
What is the combined incidence of peroxisomal disorders?
What is the characteristic of group 1?
reduced or absent number of peroxisomes; multiple enzymes affected
What is the characteristic of group 2?
Structurally normal peroxisomes; single enzyme deficiency
What is the prototype of group 2?
X-linked adrenoleukodystrophy
What is the most common peroxisomal disorder?
X-linked adrenoleukodystrophy
What is the characteristic of group 3?
abnormal structure; multipe enzymes affected