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Muscle carnitine deficiency

Fatty acid metabolism in cultured fibroblasts and muscle cells

Laboratory of Cellular Metabolism (Dr. Avigan), National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, and the Department of Neurology (Drs. Askanas and Engel), USC Neuromuscular Center, University of Southern California School of Medicine, Los Angeles, CA.

L-carnitine, as little as 25nM, greatly increased oxidation of palmitate by carnitine-depleted cultured human skin fibroblasts from normal subjects, and from two patients with muscle carnitine deficiency. Carnitine stimulated oxidation of labeled palmitate by cultured muscle cells from rat, normal humans, or patients with muscle carnitine deficiency. Carnitine reduced incorporation of palmitate into glycerides in normal fibroblasts, and it may thereby counteract cellular accumulation of glycerides in cells. Fibroblasts from patients with muscle carnitine deficiency took up labeled carnitine at a normal rate. Dexamethasone increased palmitate oxidation by normal human fibroblasts and muscle cells of rat, and normal and muscle carnitine-deficient humans. The results parallel the reported effectiveness of carnitine, glucocorticoids, or medium-chain triglycerides in treatment of these patients.

Address correspondence and reprint requests to Dr. Avigan, Laboratory of Cellular Metabolism, National Heart, Lung, and Blood Institute, National Institutes of Health, Building 10, Room 5N311, Bethesda, MD 20205.

This work was supported in part by the Muscular Dystrophy Association.

The results were presented in part at the thirty-third annual meeting of the American Academy of Neurology, Toronto, Ontario, Canada, April 1981.

Accepted for publication December 3, 1982.

This article has been cited by other articles:

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