TY - JOUR
T1 - Experimental lipid storage myopathy. A quantitative ultrastructural and biochemical study
AU - Brownell, A. Keith W.
AU - Engel, Andrew G.
PY - 1978/1
Y1 - 1978/1
N2 - 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.
AB - 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.
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U2 - 10.1016/0022-510X(78)90100-4
DO - 10.1016/0022-510X(78)90100-4
M3 - Article
C2 - 203664
AN - SCOPUS:0017928062
VL - 35
SP - 31
EP - 41
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
IS - 1
ER -