RGD Reference Report - The subcellular localization of phytanic acid oxidase in rat liver. - Rat Genome Database

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The subcellular localization of phytanic acid oxidase in rat liver.

Authors: Skjeldal, O H  Stokke, O 
Citation: Skjeldal OH and Stokke O, Biochim Biophys Acta. 1987 Sep 4;921(1):38-42.
RGD ID: 13831314
Pubmed: PMID:3620488   (View Abstract at PubMed)

Peroxisomal disorders (Zellweger's syndrome, neonatal adrenoleukodystrophy, infantile Refsum's syndrome, rhizomelic chondrodysplasia) show a series of enzymatic defects related to peroxisomal dysfunctions. Accumulation of phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) has been found in several of these patients, caused by a defect in the alpha-oxidation mechanism of this acid. The fact that the alpha-oxidation of phytanic acid is defective in the peroxisomal disorders as well as in classical Refsum's disease makes it likely that this oxidation normally takes place in the peroxisomes. A series of experiments preformed to localize the phytanic acid oxidase in subcellular fractions of rat liver show, however, that the alpha-oxidation of phytanic acid is a mitochondrial process. Free phytanic acid is the substrate, and the only cofactors necessary are ATP and Mg2+.

Objects referenced in this article
Gene Phyh phytanoyl-CoA 2-hydroxylase Rattus norvegicus

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