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

P. A. Watkins; S. J. Mihalik; O. H. Skjeldal

doi:10.1016/0006-291X(90)92064-7

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

P. A. Watkins, S. J. Mihalik, O. H. Skjeldal

Kennedy Krieger Institute

Research output: Contribution to journal › Article › peer-review

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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.

Original language	English (US)
Pages (from-to)	580-586
Number of pages	7
Journal	Biochemical and Biophysical Research Communications
Volume	167
Issue number	2
DOIs	https://doi.org/10.1016/0006-291X(90)92064-7
State	Published - Mar 16 1990

ASJC Scopus subject areas

Biophysics
Biochemistry
Molecular Biology
Cell Biology

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@article{3ce800a587344f669b3048887739b10d,

title = "Mitochondrial oxidation of phytanic acid in human and monkey liver: Implication that Refsum's disease is not a peroxisomal disorder",

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.",

author = "Watkins, {P. A.} and Mihalik, {S. J.} and Skjeldal, {O. H.}",

note = "Funding Information: The authors wish to thank Drs. Hugo W. Moser and Oddvar Stokke for their guidance and support. We thank Ms. Annette E. Howard for expert technical assistance, and Ms. Linda Reynolds for performing the hexosaminidase assays. We are indebted to Dr. James Sitzmann for help in obtaining human liver, and Dr. Linda Cork for providing monkey tissues. This work was supported in part by grants HD10981, HD24061 and RR05808 from the National Institutes Health.",

year = "1990",

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doi = "10.1016/0006-291X(90)92064-7",

language = "English (US)",

volume = "167",

pages = "580--586",

journal = "Biochemical and Biophysical Research Communications",

issn = "0006-291X",

publisher = "Elsevier B.V.",

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T1 - Mitochondrial oxidation of phytanic acid in human and monkey liver

T2 - Implication that Refsum's disease is not a peroxisomal disorder

AU - Watkins, P. A.

AU - Mihalik, S. J.

AU - Skjeldal, O. H.

N1 - Funding Information: The authors wish to thank Drs. Hugo W. Moser and Oddvar Stokke for their guidance and support. We thank Ms. Annette E. Howard for expert technical assistance, and Ms. Linda Reynolds for performing the hexosaminidase assays. We are indebted to Dr. James Sitzmann for help in obtaining human liver, and Dr. Linda Cork for providing monkey tissues. This work was supported in part by grants HD10981, HD24061 and RR05808 from the National Institutes Health.

PY - 1990/3/16

Y1 - 1990/3/16

N2 - 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.

AB - 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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ER -

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

Abstract

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