| propionyl |
the acyl radical of propionic acid; the thioester it forms with coenzyme A, propionyl CoA, is an intermediate in the degradation of some amino acids and in the oxidation of odd number chain length fatty acids.
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| propionyl-CoA carboxylase |
[EC 6.4.1.3] an enzyme of the ligase class that catalyzes the carboxylation of propionyl CoA to form methylmalonyl CoA; the reaction is part of the route by which three-carbon compounds from some amino acids and from odd numbered fatty acids are used as fuels. The enzyme is an oligomer comprising 4α and 4β chains and requires a biotin cofactor. Deficiency of enzyme activity due to a defect in either chain, an autosomal recessive trait, causes propionicacidemia; see also multiple carboxylase deficiency, under carboxylase.
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