Intracellular Localization of Hepatic Propionyl‐CoA Carboxylase and Methylmalonyl‐CoA Mutase in Humans and Normal and Vitamin B12 Deficient Rats
- 1 December 1975
- journal article
- Published by Wiley in British Journal of Haematology
- Vol. 31 (4), 501-513
- https://doi.org/10.1111/j.1365-2141.1975.tb00885.x
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.Keywords
This publication has 37 references indexed in Scilit:
- Subcellular distribution of methylmalonyl CoA carbonylmutase in human liver extractsBiochemical Medicine, 1973
- Abnormal Fatty Acid Metabolism in Peripheral Nerves of Patients with Pernicious AnemiaJCI Insight, 1973
- Methylmalonyl CoA mutase—A radiochromatographic assayClinica Chimica Acta; International Journal of Clinical Chemistry, 1972
- BIOCHEMICAL AND ULTRASTRUCTURAL PROPERTIES OF A MITOCHONDRIAL INNER MEMBRANE FRACTION DEFICIENT IN OUTER MEMBRANE AND MATRIX ACTIVITIESThe Journal of cell biology, 1970
- Studies in a patient with methylmalonic acidemiaThe Journal of Pediatrics, 1969
- AN ELECTRON-TRANSPORT SYSTEM ASSOCIATED WITH THE OUTER MEMBRANE OF LIVER MITOCHONDRIAThe Journal of cell biology, 1967
- METABOLISM OF METHYLMALONYL‐CoA AND THE ROLE OF BIOTIN AND B12 COENZYMES*Annals of the New York Academy of Sciences, 1964
- The Metabolism of Propionic AcidPublished by Wiley ,1964
- Distribution of Enzymes Between Subcellular Fractions in Animal TissuesPublished by Wiley ,1962
- Primary Metabolic Defect Supervening on Vitamin B12 Deficiency in the SheepNature, 1961