The effect of malate on propionate mitochondrial toxicity

Toyojiro Matsuishi, David A. Stumpf*, Kathy Chrislip

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


Propionic acidemia occasionally produces a toxic encephalopathy resembling Reye's syndrome, indicating disruption of mitochondrial metabolism. Liver mitochondria respiratory control ratios were reduced 46% by 5 mm propionate; inhibition correlated with matrix propionyl-CoA levels. l-Malate prevented the toxic effect of propionate and reduced the propionyl-CoA matrix concentration by 62%. The beneficial effect of l-malate is apparently due to stimulation of succinate efflux because the effect is blocked by benzylmalonate, an inhibitor of the dicarboxylate carrier. Matrix concentration of label from [1-14C]propionate was not affected by l-malate and/or benzylmalonate. l-Malate may be useful in the treatment of patients with propionic acidemia.

Original languageEnglish (US)
Pages (from-to)177-184
Number of pages8
JournalBiochemical Medicine and Metabolic Biology
Issue number2
StatePublished - Oct 1991

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism


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