| pyruvate kinase deficiency |
A hereditary deficiency of the enzyme pyruvate kinase. Pyruvate kinase deficiency results in hemolytic anemia.
출처: www.nutrabio.com/Definitions/definitions_p.htm
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| pyruvate |
a salt, ester, or anionic form of pyruvic acid. In biochemistry, the term is used interchangeably with pyruvic acid, even though pyruvate technically refers to the negatively charged ion. Pyruvate is the end product of glycolysis, and it in turn may be converted to lactate or acetyl CoA or to ethanol (as in yeasts).
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| pyruvate carboxylase |
[EC 6.4.1.1] an enzyme of the ligase class that catalyzes the irreversible carboxylation of pyruvate to form oxaloacetate. The enzyme is a mitochondrial protein containing a biotin prosthetic group, requiring Mg2+ or Mn2+ and acetyl CoA, and occurs in liver but not in muscle. The reaction is necessary for gluconeogenesis from lactate or amino acids forming pyruvate and also provides four-carbon compounds for the tricarboxylic acid cycle. Deficiency of the enzyme, an autosomal recessive trait, causes severe psychomotor retardation and lactic acidosis in infants; there is a particularly severe, rapidly fatal form, in which hyperammonemia, citrullinemia, and hyperlysinemia are also present.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| pyruvate decarboxylase |
1. [EC 4.1.1.1] an enzyme of the lyase class that catalyzes the decarboxylation of 2-keto acids to form aldehydes, part of the anaerobic fermentation pathway that produces ethanol and CO2 from glucose. The enzyme occurs in yeast and requires thiamine pyrophosphate as a cofactor. 2. formerly used to describe the enzyme now properly called pyruvate dehydrogenase (lipoamide).
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| pyruvate dehydrogenase (lipoamide) |
[EC 1.2.4.1] an enzyme of the oxidoreductase class that is a component of the multienzyme pyruvate dehydrogenase complex (q.v.). The enzyme catalyzes the oxidative decarboxylation of pyruvate, forming acetyl bound to the cofactor thiamine pyrophosphate; the acetyl is subsequently transferred to lipoamide to form acetyldihydrolipoamide, an intermediate in the overall reaction catalyzed by the complex. Deficiency of the enzyme causes lacticacidemia, ataxia, psychomotor retardation, and sometimes lactic acidosis.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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