The Subcellular Localization of Phytanic Acid Oxidase in Rat Liver

Overview

Journal Biochim Biophys Acta

Specialties Biochemistry
Biophysics

Date 1987 Sep 4

PMID 3620488

Citations 6

Authors

O H Skjeldal

O Stokke

Affiliations

Soon will be listed here.

Abstract

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+.

Citing Articles

Biochemistry of peroxisomes in health and disease.

Singh I Mol Cell Biochem. 1997; 167(1-2):1-29.

PMID: 9059978 DOI: 10.1023/a:1006883229684.

Formic acid is a product of the alpha-oxidation of fatty acids by human skin fibroblasts: deficiency of formic acid production in peroxisome-deficient fibroblasts.

Poulos A, Sharp P, Singh H, Johnson D, Carey W, Easton C Biochem J. 1993; 292 ( Pt 2):457-61.

PMID: 8503880 PMC: 1134231. DOI: 10.1042/bj2920457.

Comparison of fatty acid alpha-oxidation by rat hepatocytes and by liver microsomes fortified with NADPH, Fe3+ and phosphate.

Huang S, Van Veldhoven P, Asselberghs S, Eyssen H, de Hoffmann E, Mannaerts G Lipids. 1994; 29(10):671-8.

PMID: 7861933 DOI: 10.1007/BF02538910.

Impaired degradation of phytanic acid in cells from patients with mitochondriopathies: evidence for the involvement of ETF and the respiratory chain in phytanic acid alpha-oxidation.

Fingerhut R, Schmitz W, Garavaglia B, Reichmann H, Conzelmann E J Inherit Metab Dis. 1994; 17(5):527-32.

PMID: 7837758 DOI: 10.1007/BF00711585.

Phytanic acid alpha-oxidation and complementation analysis of classical Refsum and peroxisomal disorders.

Poll-The B, Skjeldal O, Stokke O, Poulos A, Demaugre F, Saudubray J Hum Genet. 1989; 81(2):175-81.

PMID: 2463966 DOI: 10.1007/BF00293897.