TY - JOUR
T1 - Experimental lipid storage myopathy. A quantitative ultrastructural and biochemical study
AU - Brownell, A. Keith W.
AU - Engel, Andrew G.
N1 - Funding Information:
Since the discovery ofcarnitine deficiency as a cause of human triglyceride storage myopathy (Engel and Angelini 1973), metabolic abnormalities which result in lipid accumulation in muscle have been viewed with increasing interest. The spectrum of clinical abnormalities resulting from carnitine deficiency has been recently reviewed (Engel and Banker 1977). Impaired oxidation of long-chain fatty acids due to a de- * Present address : Faculty of Medicine, University of Calgary, Calgary, Canada. Reprint requests to Dr. Engel. This work was supported in part by Research Grant NS-6277 from the National Institutes of Health, U.S. Public Health Service and by a Research Center Grant from the Muscular Dystrophy Association of America, Inc. During the conduct of this work, Dr. Brownell was recipient of a Fellowship from the Medical Research Council of Canada.
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.
UR - http://www.scopus.com/inward/record.url?scp=0017928062&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0017928062&partnerID=8YFLogxK
U2 - 10.1016/0022-510X(78)90100-4
DO - 10.1016/0022-510X(78)90100-4
M3 - Article
C2 - 203664
AN - SCOPUS:0017928062
SN - 0022-510X
VL - 35
SP - 31
EP - 41
JO - Journal of the neurological sciences
JF - Journal of the neurological sciences
IS - 1
ER -