The inhibition by methylmalonic acid of malate transport by the dicarboxylate carrier in rat liver mitochondria: A possible explanation for hypoglycemia in methylmalonic aciduria

AUTOR(ES)

Halperin, M. L.

RESUMO

We report the effects of methylmalonic acid (MMA) on the mitochondrial transport systems for malate, α-oxoglutarate, and isocitrate. MMA is shown to be a substrate for all three carrier systems, and an inhibitor of the malate-phosphate exchange carrier. The effects of MMA on the metabolism of malate, oxoglutarate, and isocitrate by rat liver mitochondria are demonstrated to be mediated by the influence of MMA on the transport step. A hypothesis regarding the metabolic impairments responsible for hypoglycemia and ketonemia in methylmalonic aciduria is formulated in relation to these findings.

Documentos Relacionados

• Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia.
• Effects of Palmitoyl CoA on Citrate and Malate Transport by Rat Liver Mitochondria
• DNA biosynthesis in mitochondria: partial purification of a distinct DNA polymerase from isolated rat liver mitochondria.
• Transport of proteins into mitochondria: a potassium diffusion potential is able to drive the import of ADP/ATP carrier.
• Chronic antigenic stimuli as a possible explanation for the immunodepression caused by liver cirrhosis