1962
DOI: 10.1016/s0076-6879(62)05279-9
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[78] Mitochondrial propionyl carboxylase

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Cited by 17 publications
(9 citation statements)
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“…The metabolic role for this mitochondrial enzyme is thought to be the removal of malonyl CoA produced by the action of propionyl CoA carboxylase on acetyl CoA in the mitochondria [3]. When the decarboxylase activity is deficient, malonyl CoA accumulates in the mitochondria with a portion transesterified to malonylcarnitine by carnitine palmitoyltransferase II and a portion hydrolyzed to free malonic acid leading to urinary excretion of malonylcarnitine and malonic acid.…”
Section: Resultsmentioning
confidence: 99%
“…The metabolic role for this mitochondrial enzyme is thought to be the removal of malonyl CoA produced by the action of propionyl CoA carboxylase on acetyl CoA in the mitochondria [3]. When the decarboxylase activity is deficient, malonyl CoA accumulates in the mitochondria with a portion transesterified to malonylcarnitine by carnitine palmitoyltransferase II and a portion hydrolyzed to free malonic acid leading to urinary excretion of malonylcarnitine and malonic acid.…”
Section: Resultsmentioning
confidence: 99%
“…Propionyl CoA carboxylase was assayed by the l4CO2-fixation method of Lane and Halenz (12) except that the reaction was stopped after 8 min with 0.3 ml of 20% trichloroacetic acid.…”
mentioning
confidence: 99%
“…The incorporation of this compound into malonic acid is dependent upon the carboxylation of acetyl CoA and the subcellular distribution of the enzyme defect indicates that this is occurring within the mitochondrion. Acetyl CoA carboxylase is not present within the mitochondrion (Hiilsmann, 1966), however propionyl CoA carboxylase is located within this organelle (Scholte, 1969) and does carboxylate acetyl CoA at a slow rate compared to the true substrate, propionyl CoA (Lane et al, 1960). No evidence was obtained in this study to support the suggestion that malonic acid can be formed from propionyl CoA (Rosenberg, 1983).…”
Section: Discussionmentioning
confidence: 58%