| 3-methyl-2-oxobutanoate dehydrogenase (lipoamide) |
[EC 1.2.4.4] an enzyme of the oxidoreductase class that is a component of the multienzyme branched-chain α-keto acid dehydrogenase complex (q.v.). The enzyme catalyzes the oxidative decarboxylation of the branched chain amino acids leucine, isoleucine, and valine, transferring the products formed to the lipoic acid moiety of dihydrolipoamide acyltransferase via a thiamine pyrophosphate cofactor. See also maple syrup urine disease, under disease. Called also α-ketoisovalerate dehydrogenase.
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| 3-methylcholanthrene |
a highly carcinogenic polycyclic aromatic hydrocarbon synthesized by pyrolytic degradation of cholic acid, deoxycholic acid, or cholesterol. It is a procarcinogen that requires metabolic activation to exert a mutagenic effect and is widely used in laboratory studies of chemical carcinogenesis. Abbreviated MCA.
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| 3-methylcrotonic acid |
crotonic acid methylated at the 3 carbon; it is excreted at elevated levels in urine when 3-methylcrotonoyl-CoA carboxylase activity is impaired.
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| 3-methylcrotonyl |
the radical of 3-methylcrotonic acid; the thioester formed with coenzyme A, 3-methylcrotonyl CoA, is an intermediate in the degradation of leucine.
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| 3-methylcrotonyl CoA carboxylase deficiency |
an autosomal recessive aminoacidopathy due to deficiency of methylcrotonoyl-CoA carboxylase, characterized by increased urine levels of 3-methylcrotonylglycine, 3-methylcrotonic acid, and 3-hydroxyisovaleric acid and variable presentation of mental retardation, central nervous system dysfunction, and muscular atrophy. Written also β-methylcrotonyl CoA carboxylase deficiency.
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