Experimental lipid storage myopathy. A quantitative ultrastructural and biochemical study

A. Keith W. Brownell, Andrew G. Engel

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

A lipid storage myopathy was induced in rats treated with daily doses of 2.5 g/kg brominated vegetable oil. As in human lipid storage myopathies, type I fibers were selectively severely affected, the lipid deposits were surrounded by mitochondria and the mitochondrial fraction of the affected fibers was increased. The oxidation of [U-14C]palmitate, [1-14C]octanoate and β-[3-14C]hydroxybutyrate was significantly depressed but [1-14C]palmitate, as well as labeled pyruvate and succinate were oxidized at normal rates. The activities of long-chain, medium-chain and short-chain carnitine acyltransferases and the muscle carnitine levels were normal. The lipid storage is attributed to impaired beta oxidation of medium-chain and short-chain fatty acyl residues. An approach to the investigation of those human lipid storage myopathies not due to defects in the carnitine system is suggested.

Original languageEnglish (US)
Pages (from-to)31-41
Number of pages11
JournalJournal of the neurological sciences
Volume35
Issue number1
DOIs
StatePublished - Jan 1978

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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