| beta-aminoisobutyrate:pyruvate aminotransferase | Beta-aminosiobutyrate:pyruvate transaminase;an enzyme that catalyses the reversible transfer of an amino group from beta-aminoisobutyrate to pyruvate, producing l-alanine and methylmalonate saemialdehyde. A step in valine degradation. A deficiency of beta-aminoisobutyrate:pyruvate aminotransferase results in hyper-beta-aminoisobutyric aciduria. (05 Mar 2000) |
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| valine-pyruvate transaminase | <enzyme> E coli enzyme catalyzing the terminal step of valine biosynthesis; consider also EC 2.6.1.42, branched-chain-amino-acid transaminase; alanine-alpha-oxoisovalerate aminotransferase and alanine-alpha-ketoisovalerate aminotransferase were ens to alanine aminotransferase 1981-93 Registry number: EC 2.6.1.66 Synonym: alanine-valine transaminase, transaminase c, alanine alpha-ketoisovalerate aminotransferase, alanine-alpha-oxoisovalerate aminotransferase, alanine-alpha-ketoisovalerate aminotransferase (26 Jun 1999) |
| glutamine-pyruvate aminotransferase | <enzyme> Consider also glutamine transaminase k if cysteine conjugate beta-lyase activity is also present; l-methionine can act as donor; glyoxylate can act as acceptor Registry number: EC 2.6.1.15 Synonym: glutamine alpha-ketoacid transaminase, glutamine transaminase, glutamine transaminase l, glutamine oxo-acid aminotransferase, glutaminase II, glutamine aminotransferase, l-methionine aminotransferase (26 Jun 1999) |
| chorismate pyruvate-lyase | <enzyme> Forms 4-hydroxybenzoic acid for ubiquinone biosynthesis; chorismate pyruvate-lyase (amino-accepting) is anthranilate synthase Registry number: EC 4.1.3.- Synonym: ubic gene product, chorismate lyase (26 Jun 1999) |
| serine-pyruvate aminotransferase | <enzyme> Do not confuse with alanine-glyoxylate aminotransferase (EC 2.6.1.44), even though they may be products of the same gene; index whichever is discussed or both Registry number: EC 2.6.1.51 Synonym: serine aminotransferase, serine-pyruvate transaminase (26 Jun 1999) |
| pyruvate | <biochemistry> Pyruvate is the final product of glycolysis. You get two molecules of pyruvate for every molecule of glucose that goes through glycolysis. (22 Aug 1998) |
| pyruvate carboxylase | <enzyme> An enzyme that catalyses the formation of oxaloacetate from pyruvate, carbon dioxide and ATP in gluconeogenesis. (18 Nov 1997) |
| pyruvate carboxylase deficiency | An autosomal recessive pyruvate metabolism disorder resulting from absent or deficient expression of pyruvate carboxylase activity. Decreased production of oxaloacetate leads to decreased gluconeogenesis, thereby causing fasting hypoglycaemia, lactic acid acidosis, and decreased synthesis of amino acid neurotransmitters. Clinical presentations include acidosis, ataxia, mental retardation; sometimes co-occurs with leigh disease. (12 Dec 1998) |
| pyruvate decarboxylase | <enzyme> Catalyses the decarboxylation of an alpha keto acid to an aldehyde and carbon dioxide. Thiamine pyrophosphate is an essential cofactor. In lower organisms, which ferment glucose to ethanol and carbon dioxide, the enzyme irreversibly decarboxylates pyruvate to acetaldehyde. Registry number: EC 4.1.1.1 (12 Dec 1998) |
| pyruvate dehydrogenase | <enzyme> A complex multienzyme system that catalyses the conversion of (pyruvate + CoA + NAD) to (acetyl CoA + CO2 + NAD). (18 Nov 1997) |
| pyruvate dehydrogenase complex | <enzyme> A complex multienzyme system that catalyses the conversion of (pyruvate + CoA + NAD) to (acetyl CoA + CO2 + NAD). (18 Nov 1997) |
| pyruvate dehydrogenase complex deficiency | An autosomal recessive pyruvate metabolism disorder resulting from deficient enzyme activity in one of several proteins of pyruvate dehydrogenase complex, resulting in deficiency of acetyl CoA. Deficiency in acetyl CoA product reduces the synthesis of acetylcholine, thereby causing neurological abnormalities. Clinical presentations include lactic acidosis, mental retardation, and ataxia. (12 Dec 1998) |
| pyruvate dehydrogenase complex inactivase | <enzyme> Pharmacological action: enzyme inhibitor Registry number: EC 3.4.- (26 Jun 1999) |
| pyruvate dehydrogenase (cytochrome) | An oxidoreductase catalyzing reaction between ferricytochrome b1 and pyruvate to yield acetate and CO2, and ferrocytochrome b1. (05 Mar 2000) |
| pyruvate dehydrogenase (lipoamide) | An oxidoreductase catalyzing conversion of pyruvate and (oxidised) lipoamide to CO2 and S6-acetyldihydrolipoamide in two successive reactions: the first between pyruvate and thiamin pyrophosphate to yield CO2 and alpha-hydroxyethylthiamin pyrophosphate (active pyruvate); the second between the last named and lipoamide to regain the thiamin pyrophosphate and yield S6-acetylhydrolipoamide. Compare: alpha-ketodecarboxylase. (05 Mar 2000) |