| pyruvate dehydrogenase c. |
a multienzyme complex consisting of at least three distinct enzymes: pyruvate dehydrogenase (lipoamide) [EC 1.2.4.1], dihydrolipoamide S-acetyltransferase [EC 2.3.1.12], and dihydrolipoamide dehydrogenase [EC 1.8.1.4]. The integrated enzyme complex requires the cofactors thiamine pyrophosphate, lipoic acid, coenzyme A, FAD, and NAD+. It catalyzes the formation of acetyl coenzyme A from pyruvate and coenzyme A, using NAD+ as an electron acceptor; the acetyl coenzyme A is used in fatty acid synthesis, for acetylations, and for oxidation via the tricarboxylic acid cycle. Deficiency of any component of the complex results in lacticacidemia, ataxia, and psychomotor retardation. See also lipoamide dehydrogenase deficiency.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| pyruvate dehydrogenase complex |
see under complex.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| pyruvate kinase |
[EC 2.7.1.40] an enzyme of the transferase class that catalyzes the transfer of high energy phosphate from phosphoenolpyruvate to ADP to yield ATP and pyruvate; it is one of two reactions generating ATP in the Embden-Meyerhof pathway (see illustration at pathway) and a key regulatory site in this pathway. The enzyme has three distinct isozymes. Deficiency of pyruvate kinase activity in erythrocytes, an autosomal recessive trait, results in hemolytic anemia.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| pyruvate kinase (PK) deficiency |
deficiency of the erythrocytic isozyme of pyruvate kinase, the most common glycolytic enzyme defect in the Embden-Meyerhof pathway; deficient product (ATP) causes chronic hemolytic anemia of widely variable severity. PK deficiency is an autosomal recessive trait and has no distinguishing clinical features from the other hemolytic disorders.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| pyruvate kinase deficiency |
hereditary blood disorder characterized by a deficiency of the enzyme pyruvate kinase. physical findings associated with the disorder may include reduced levels of oxygen-carrying hemoglobulin in the blood due to premature destruction of red blood cells (hemolytic anemia); abnormally increased levels of bilirubin in the blood (hyperbilirubinemia); abnormal enlargement of the spleen (splenomegaly); and/or other abnormalities.
출처: ipp.boku.ac.at/pz/ref/glossar1.html
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