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Biochimie. 2014 Mar;98:4-15. doi: 10.1016/j.biochi.2013.09.019. Epub 2013 Sep 26.

The human peroxisome in health and disease: the story of an oddity becoming a vital organelle.

Author information

1
INSERM, Laboratory of Biochemistry and Molecular Biology, Hormonology-Metabolism-Nutrition-Oncology, Centre of Biology and Pathology (CBP), CHU Lille, France. Electronic address: joseph.vamecq@inserm.fr.
2
Laboratory of Biochemistry of Peroxisome, Inflammation & Lipids Metabolism (BioPeroxIL-EA7270), University of Burgundy, 21000 Dijon, France.

Abstract

Since the first report by Rhodin in 1954, our knowledge on mammalian microbodies/peroxisomes has known several periods. An initial two decades period (1954-1973) has contributed to the biochemical individualisation of peroxisomes as a new class of subcellular organelles (de Duve, 1965). The corresponding research period failed to define a clear role of mammalian peroxisomes in vital functions and intermediary metabolism, explaining why feeling that peroxisomes might be in the human cell oddities has prevailed during several decades. The period standing from 1973 to nowadays has progressively removed this cell oddity view of peroxisomes by highlighting vital function and metabolic role of peroxisomes in health and disease along with genetic and metabolic regulation of peroxisomal protein content, organelle envelope formation and protein signal targeting mechanisms. Research on peroxisomes and their response to various drugs and metabolites, dietary and physiological conditions has also played a key role in the discovery of peroxisome proliferator activated receptors (PPARs) belonging to the nuclear hormone receptor superfamily and for which impact in science and medicine goes now by far beyond that of the peroxisomes.

KEYWORDS:

Bile acids; Hydrogen peroxide-producing oxidases; PEX; PPARs; Peroxisomal disorders; Peroxisomal β-oxidation; Peroxisome biogenesis; Peroxisomes; Plasmalogens

PMID:
24075875
DOI:
10.1016/j.biochi.2013.09.019
[Indexed for MEDLINE]

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