Comparison between the formation and the oxidation of dicarboxylylcarnitine esters in rat liver and skeletal muscle: possible implications for human inborn disorders of mitochondrial beta-oxidation

Journal of Inherited Metabolic Disease
J Vamecq, J P Draye

Abstract

The activity of dicarboxylyl-CoA synthetase previously reported in rat liver was detected, in the presence of added detergents, in rat skeletal muscle. In both tissues, carnitine dicarboxylyltransferase activities were recorded but their substrate chain length specificity was, however, different. In rat skeletal muscle, but not liver, a carnitine-dependent oxidation by intact mitochondria of dodecanedioyl-CoA was easily detected by the spectrophotometric measurement of the substrate-dependent ferricyanide reduction. The implications of the present data for the pathogenesis of disorders with excess urine dicarboxylylcarnitine esters are discussed.

Citations

Oct 1, 1994·European Journal of Pediatrics·A C SewellR J Wanders
Jul 10, 1998·Clinica Chimica Acta; International Journal of Clinical Chemistry·M FontaineJ Vamecq
May 1, 1994·JPEN. Journal of Parenteral and Enteral Nutrition·G MingroneM Castagneto
Apr 29, 1998·Cancer Investigation·S Manne
Jun 1, 1995·Clinical Nutrition : Official Journal of the European Society of Parenteral and Enteral Nutrition·A V Grego, G Mingrone

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