NDLI: Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Van Veldhoven, Paul P.
Description	Country affiliation: Belgium Author Affiliation: Van Veldhoven PP ( Katholieke Universiteit Leuven, Department of Molecular Cell Biology, LIPIT, Campus Gasthuisberg, Herestraat, Leuven, Belgium. paul.vanveldhoven@med.kuleuven.be)
Abstract	In humans, peroxisomes harbor a complex set of enzymes acting on various lipophilic carboxylic acids, organized in two basic pathways, alpha-oxidation and beta-oxidation; the latter pathway can also handle omega-oxidized compounds. Some oxidation products are crucial to human health (primary bile acids and polyunsaturated FAs), whereas other substrates have to be degraded in order to avoid neuropathology at a later age (very long-chain FAs and xenobiotic phytanic acid and pristanic acid). Whereas total absence of peroxisomes is lethal, single peroxisomal protein deficiencies can present with a mild or severe phenotype and are more informative to understand the pathogenic factors. The currently known single protein deficiencies equal about one-fourth of the number of proteins involved in peroxisomal FA metabolism. The biochemical properties of these proteins are highlighted, followed by an overview of the known diseases.
File Format	HTM / HTML
ISSN	00222275
e-ISSN	15397262
DOI	10.1194/jlr.R005959
Journal	The Journal of Lipid Research
Issue Number	10
Volume Number	51
Language	English
Publisher	American Society for Biochemistry and Molecular Biology
Publisher Date	2010-10-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Discipline Biochemistry Fatty Acids, Unsaturated Metabolism Fatty Acids Peroxisomal Disorders Genetics Peroxisomes Enzymology Phytanic Acid Animals Lipid Metabolism Physiology Oxidation-reduction Research Support, Non-u.s. Gov't
Content Type	Text
Resource Type	Article
Subject	Cell Biology Biochemistry Endocrinology

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Phytanic acid metabolism in health and disease

Bile acids: the role of peroxisomes.

Degradation of very long chain dicarboxylic polyunsaturated fatty acids in mouse hepatocytes, a peroxisomal process

Very-long-chain polyunsaturated fatty acids accumulate in phosphatidylcholine of fibroblasts from patients with Zellweger syndrome and acyl-CoA oxidase1 deficiency

Hepatic oleate regulates adipose tissue lipogenesis and fatty acid oxidation.

Fatty acid metabolism, energy expenditure and insulin resistance in muscle.

Peroxisomes, lipid metabolism and lipotoxicity

Transfer of metabolites across the peroxisomal membrane☆

FATP2 is a hepatic fatty acid transporter and peroxisomal very long-chain acyl-CoA synthetase.

Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism.

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Degradation of very long chain dicarboxylic polyunsaturated fatty acids in mouse hepatocytes, a peroxisomal process

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