Intracellular Localization of Hepatic Propionyl‐CoA Carboxylase and Methylmalonyl‐CoA Mutase in Humans and Normal and Vitamin B12 Deficient Rats

E. P. Frenkel, R. L. Kitchens

Research output: Contribution to journalArticle

15 Scopus citations

Abstract

The intracellular localization of the enzymes in the vitamin B12 dependent pathway which involves the oxidation of propionate was studied in rat liver obtained from normal and vitamin B12 deficient rats as well as from man. The subcellular site of propionyl CoA carboxylase and the vitamin B12 dependent‐methylmalonyl CoA mutase were determined. All of the activity of these two enzymes was demonstrated to be in the mitochondria and those enzymes were shown to be loosely bound to the inner membrane‐matrix portion of the mitochondria. Vitamin B12 deficiency did not alter the subcellular localization. Finally, a rapid enzymatic assay for methylmalonyl‐CoA mutase was described.

Original languageEnglish (US)
Pages (from-to)501-513
Number of pages13
JournalBritish Journal of Haematology
Volume31
Issue number4
DOIs
StatePublished - Dec 1975

ASJC Scopus subject areas

  • Hematology

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