[Effect of L-carnitine on metabolic disorders in rats with experimental acyl-CoA dehydrogenase deficiency]

Effect of L-carnitine (LC) on the metabolism of organic acids and carnitine homeostasis was studied in rats with riboflavin deficiency producing unusual dicarboxylic acidurea and modeling multiple acyl-CoA dehydrogenase deficiency in humans. Riboflavin deficient (RFD) rats exhibited increased excretion of glutaric, ethylmalonic, and methylsuccinic acids, as well as isovaleryl-, butyryl-, isobutyryl-, 2-methyl-butyryl-, and hexanoylglycine, short-chain and medium-chain saturated, and unsaturated dicarboxylic organic acids (C6-C10). RFD rats also showed a decrease in the concentration of free LC in the blood plasma and in tissues, an increase in the level of isobutyryl- and isovalerylcarnitine in muscle tissue, and reduction in the level of acetyl- and propionylcarnitine in the blood plasma, kidney, and liver (all changes detected relative to animals in the control group). The introduction of LC to RFD rats normalized the LC homeostasis by increasing free LC concentration in the blood plasma and tissues, enhanced the acyl-LC excretion with urine and the level in tissues, and reduced the manifestations of organic acidurea.