| pyruvate carboxylase | <enzyme> An enzyme that catalyses the formation of oxaloacetate from pyruvate, carbon dioxide and ATP in gluconeogenesis. (18 Nov 1997) |
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| 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-formate-lyase-deactivase | <chemical> Encoded by adhe gene product from E coli; pfla (radical form) yields pfl (non-radical form) in presence of fe++, nad and CoA and the multienzyme complex consisting of alcohol plus acetaldehyde-CoA dehydrogenase activities Synonym: pfl-deactivase, adhe multienzyme, adhe gene product (26 Jun 1999) |
| pyruvate kinase | <enzyme> ATP:pyruvate 2-o-phosphotransferase. A phosphotransferase that catalyses reversibly the phosphorylation of pyruvate to phosphoenolpyruvate in the presence of ATP. It has four isozymes (l, r, m1, and m2). Deficiency of the enzyme results in haemolytic anaemia. Registry number: EC 2.7.1.40 (12 Dec 1998) |
| pyruvate kinase deficiency | A disorder in which there is a deficiency of pyruvate kinase in red blood cells; characterised by haemolytic anaemia varying in degree from one patient to another; autosomal recessive inheritance. (05 Mar 2000) |
| pyruvate kinase phosphatase | <enzyme> Reactivates pyruvate kinase Registry number: EC 3.1.3.- (26 Jun 1999) |
| pyruvate metabolism, inborn errors | Hereditary disorders of pyruvate metabolism. They are difficult to diagnose and describe because pyruvate is a key intermediate in glycolysis, gluconeogenesis, and the tricarboxylic acid cycle. Some inherited metabolic disorders may alter pyruvate metabolism indirectly. Disorders in pyruvate metabolism appear to lead to deficiencies in neurotransmitter synthesis and, consequently, to nervous system disorders. (12 Dec 1998) |
| pyruvate, orthophosphate dikinase | <enzyme> An enzyme that catalyses the reaction of ATP, pyruvate, and orthophosphate to form AMP plus phosphoenolpyruvate plus pyrophosphate. Chemical name: ATP:pyruvate, orthophosphate phosphotransferase Registry number: EC 2.7.9.1 (12 Dec 1998) |
| pyruvate oxidase | <enzyme> Registry number: EC 1.2.3.3 (12 Dec 1998) |
| pyruvate oxidation factor | <biochemistry> 1,2 dithiolane 3 valeric acid. Regarded as a coenzyme in the oxoglutarate dehydrogenase complex of the citric acid cycle. Involved generally in oxidative decarboxylations of _ keto acids. A growth factor for some organisms. (18 Nov 1997) |
| enol pyruvate | CH2==C(OH)-COO-un, the form of pyruvate encountered in the biologically important phosphoenolpyruvate (enol pyruvate phosphate), not in the free form. (05 Mar 2000) |
| ketal pyruvate transferase | <enzyme> Involves the transfer of the pyruvyl residue from phosphoenolpyruvate to the external mannose of pentasaccharide diphosphate lipid Registry number: EC 2.3.1.- (26 Jun 1999) |
| kynurenine-pyruvate aminotransferase | <enzyme> Found in rat kidney and brain; forms kynurenic acid from kynurenine; pyruvate, 2-keto-n-valerate, 2-keto-n-caproate, 2-keto-isocaproate and oxaloacetate are good acceptors Registry number: EC 2.6.1.- (26 Jun 1999) |
| core II protein, ubiquinol-cytochrome c reductase | <chemical> Member of the mitochondrial-protein-processing family; protein found in subunits of ubiquinol-cytochrome c reductase; amino acid sequence given in first source Synonym: core II protein, uccreductase (26 Jun 1999) |