Journal
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
Volume 42, Issue 11, Pages 1771-1774Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.biocel.2010.07.004
Keywords
Peroxisome trafficking; Protein translocation; Organelle biogenesis; Receptor cycling; Peroxin
Categories
Funding
- Netherlands Organisation for Scientific Research (NWO)
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Peroxins are proteins that regulate the biogenesis of peroxisomes-small vesicular subcellular organelles essential for human life and health. A key peroxin - to date the best studied - is peroxin 5. Structurally, peroxin 5 is a bi-domain protein of about 70 kDa containing both globular and non-globular segments and displaying conformational flexibility. Functionally, it is a cycling receptor for importing essential enzymes into the peroxisome lumen, facilitated by highly promiscuous interactions with numerous proteins and possibly lipids. Peroxin 5 has medical significance in that (i) congenital defects can lead to fatal peroxisome biogenesis disorders, (ii) inefficient peroxisome targeting is linked to disease and aging and (iii) differences between human peroxin 5 and homologues in pathogens may be exploited in the development of therapeutics. (C) 2010 Elsevier Ltd. All rights reserved.
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