Tissue distribution of phytanic acid and its analogues in a kinship with Refsum's disease

J. K. Yao, Peter J Dyck

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Three of six kin were identified, by high performance thin layer chromatography, capillary gas chromatography and mass spectrometry, as having phytanic acid storage disease. Phytanic acid was found in triacylglycerol and, to a lesser degree, in phosphatidylcholine and free fatty acids. An unsaturated analogue of phytanic acid was additionally identified in plasma and erythrocyte triacylglycerols. In plasma, branched chain fatty acids were primarily localized in the low density lipoprotein fraction. The concentration of plasma major fatty acids was not affected by the presence of these branched chain fatty acids. In contrast to plasma, only small amounts of phytanic acid were found in cerebrospinal fluid and biopsied sural nerve. The nerve phytanate was mainly associated with triacylglycerol in epineurial and perineurial tissues. Lack of phytanate accumulation in sural endoneurium, even in cases with severe fiber degeneration, suggests that demyelination in Refsum's disease may not be due to myelin instability resulting from the incorporation of branched chain fatty acids into peripheral nerve membrane.

Original languageEnglish (US)
Pages (from-to)69-75
Number of pages7
JournalLipids
Volume22
Issue number2
StatePublished - 1987

Fingerprint

Refsum Disease
Phytanic Acid
kinship
tissue distribution
Tissue Distribution
branched chain fatty acids
Fatty Acids
Tissue
Plasmas
Triglycerides
peripheral nerves
acids
triacylglycerols
Peripheral Nerves
nerve tissue
Cerebrospinal fluid
Thin layer chromatography
Sural Nerve
capillary gas chromatography
myelin sheath

ASJC Scopus subject areas

  • Food Science
  • Biochemistry, Genetics and Molecular Biology(all)
  • Biochemistry
  • Medicine (miscellaneous)

Cite this

Tissue distribution of phytanic acid and its analogues in a kinship with Refsum's disease. / Yao, J. K.; Dyck, Peter J.

In: Lipids, Vol. 22, No. 2, 1987, p. 69-75.

Research output: Contribution to journalArticle

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