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Biochimie. 2014 Mar;98:36-44. doi: 10.1016/j.biochi.2013.08.022. Epub 2013 Sep 3.

Metabolic functions of peroxisomes in health and disease.

Author information

1
University of Amsterdam, Academic Medical Center, Departments of Clinical Chemistry and Pediatrics, Emma Children's Hospital, Laboratory Genetic Metabolic Diseases, Room F0-226, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. Electronic address: r.j.wanders@amc.uva.nl.

Abstract

Peroxisomes are subcellular organelles which are present in virtually every eukaryotic cell and catalyze a large number of metabolic functions. The importance of peroxisomes for humans is stressed by the existence of a large group of genetic diseases in which either the biogenesis of peroxisomes is impaired or one of its metabolic functions. Thanks to the work on Zellweger syndrome which is the prototype of the group of peroxisomal disorders, much has been learned about the metabolism and biogenesis of peroxisomes in humans. These metabolic functions include: (1.) fatty acid beta-oxidation; (2.) etherphospholipid biosynthesis; (3.) fatty acid alpha-oxidation, and (4.) glyoxylate detoxification. Since peroxisomes lack a citric acid cycle and a respiratory chain, peroxisomes are relatively helpless organelles which rely heavily on their cross-talk with other subcellular organelles in order to metabolize the end products of metabolism as generated in peroxisomes. The metabolic functions of peroxisomes in humans will be briefly described in this review with emphasis on the cross-talk with other subcellular organelles as well as the peroxisomal disorders in which one or more peroxisomal functions are impaired.

KEYWORDS:

Endoplasmic reticulum; Fatty acid; Peroxisomal disorders; Peroxisomes; Zellweger syndrome

PMID:
24012550
DOI:
10.1016/j.biochi.2013.08.022
[Indexed for MEDLINE]

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