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CancerWEB ¿µ¿µ ÀÇÇлçÀü ¸ÂÃã °Ë»ö °á°ú : 15 ÆäÀÌÁö: 19
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 (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)
pyruvate dehydrogenase (lipoamide)-phosphatase <enzyme> (pyruvate dehydrogenase (lipoamide))-phosphate phosphohydrolase. A mitochondrial enzyme that catalyses the hydrolytic removal of a phosphate on a specific seryl hydroxyl group of pyruvate dehydrogenase, reactivating the enzyme complex.
Registry number: EC 3.1.3.43
(12 Dec 1998)
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 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 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)
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pyramidal e. eminentia pyramidalis.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
pyramidal f. Le Fort II f.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
pyramidal f. of medulla oblongata tractus pyramidalis (def. 1).
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
pyramid of the cerebellum A median ventral projection of the vermis of the cerebellum lying between the tuber and uvula.
Ãâó:
pyramidal l. of cerebral cortex, external lamina pyramidalis externa.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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