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. 1971 Nov;50(11):2276–2282. doi: 10.1172/JCI106725

The inhibition by methylmalonic acid of malate transport by the dicarboxylate carrier in rat liver mitochondria

A possible explanation for hypoglycemia in methylmalonic aciduria

M L Halperin 1,2, C M Schiller 1,2, I B Fritz 1,2
PMCID: PMC292169  PMID: 4398537

Abstract

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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