Literature DB >> 1690986

Mitochondrial oxidation of phytanic acid in human and monkey liver: implication that Refsum's disease is not a peroxisomal disorder.

P A Watkins1, S J Mihalik.   

Abstract

The subcellular site of oxidation of [1-14C]phytanic acid to 14CO2 was investigated in human and monkey liver. In both species, this activity was associated with fractions enriched in mitochondria. Fractions enriched in peroxisomes had no detectable phytanic acid oxidase activity. The mitochondrial inhibitors antimycin A and rotenone significantly decreased 14CO2 production in mitochondria-rich fractions from human and monkey liver. These inhibitors also blocked phytanic acid oxidation in cultured human skin fibroblasts. These data suggest that alpha-oxidation of phytanic acid is a mitochondrial rather than a peroxisomal process in primates.

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Year:  1990        PMID： 1690986     DOI： 10.1016/0006-291x(90)92064-7

Source DB:  PubMed          Journal:  Biochem Biophys Res Commun        ISSN： 0006-291X            Impact factor:   3.575


  7 in total

1.  Accumulation of phytanic acid alpha-oxidation intermediates in Zellweger fibroblasts.

Authors:  R Fingerhut; W Schmitz; E Conzelmann
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

Review 2.  Metabolic pigmentary retinopathies: diagnosis and therapeutic attempts.

Authors:  B T Poll-The; T Billette de Villemeur; M Abitbol; J L Dufier; J M Saudubray
Journal:  Eur J Pediatr       Date:  1992-01       Impact factor: 3.183

Review 3.  Biochemistry of peroxisomes in health and disease.

Authors:  I Singh
Journal:  Mol Cell Biochem       Date:  1997-02       Impact factor: 3.396

4.  Isolated defect of peroxisomal beta-oxidation in a 16-year-old patient.

Authors:  R Santer; A Claviez; H D Oldigs; J Schaub; R B Schutgens; R J Wanders
Journal:  Eur J Pediatr       Date:  1993-04       Impact factor: 3.183

Review 5.  Liver pathology and immunocytochemistry in congenital peroxisomal diseases: a review.

Authors:  F Roels; M Espeel; D De Craemer
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

6.  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.

Authors:  R Fingerhut; W Schmitz; B Garavaglia; H Reichmann; E Conzelmann
Journal:  J Inherit Metab Dis       Date:  1994       Impact factor: 4.982

7.  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.

Authors:  A Poulos; P Sharp; H Singh; D W Johnson; W F Carey; C Easton
Journal:  Biochem J       Date:  1993-06-01       Impact factor: 3.857
  7 in total

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