Py : KMLE 의학 검색 엔진 - 의학사전, 의학용어, 의학약어, 의학논문, 약품/의약품 검색

MeSH(Medical Subject Headings) 맞춤 검색 (http://www.nlm.nih.gov) 결과 : 5 페이지: 18

Pyruvate Dehydrogenase Complex - 새창 A multienzyme complex responsible for the formation of ACETYL COENZYME A from pyruvate. The enzyme components are PYRUVATE DEHYDROGENASE (LIPOAMIDE); dihydrolipoamide acetyltransferase; and LIPOAMIDE DEHYDROGENASE. Pyruvate dehydrogenase complex is subject to three types of control: inhibited by acetyl-CoA and NADH; influenced by the energy state of the cell; and inhibited when a specific serine residue in the pyruvate decarboxylase is phoshorylated by ATP. PYRUVATE DEHYDROGENASE (LIPOAMIDE)-PHOSPHATASE catalyzes reactivation of the complex. (From Concise Encyclopedia Biochemistry and Molecular Biology, 3rd ed)
Synonyms : Complex, Pyruvate Dehydrogenase, Dehydrogenase Complex, Pyruvate
Pyruvate Dehydrogenase Complex Deficiency Disease - 새창 An inherited metabolic disorder caused by deficient enzyme activity in the PYRUVATE DEHYDROGENASE COMPLEX, resulting in deficiency of acetyl CoA and reduced synthesis of acetylcholine. Two clinical forms are recognized: neonatal and juvenile. The neonatal form is a relatively common cause of lactic acidosis in the first weeks of life and may also feature an erythematous rash. The juvenile form presents with lactic acidosis, alopecia, intermittent ATAXIA; SEIZURES; and an erythematous rash. (From J Inherit Metab Dis 1996;19(4):452-62) Autosomal recessive and X-linked forms are caused by mutations in the genes for the three different enzyme components of this multisubunit pyruvate dehydrogenase complex. One of the mutations at Xp22.2-p22.1 in the gene for the E1 alpha component of the complex leads to LEIGH DISEASE.
Synonyms : Juvenile Pyruvate Dehydrogenase Complex Deficiency Disease, Neonatal Pyruvate Dehydrogenase Complex Deficiency Disease, PDHC Deficiency Disease, Pyruvate Dehydrogenase Complex Deficiency Disease, Juvenile, Type I Ataxia with Lactic Acidosis
Pyruvate Kinase - 새창 ATP:pyruvate 2-O-phosphotransferase. A phosphotransferase that catalyzes 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 hemolytic anemia. EC 2.7.1.40.
Synonyms : L-Type Pyruvate Kinase, M-Type Pyruvate Kinase, M1-Type Pyruvate Kinase, M2-Type Pyruvate Kinase, Pyruvate Kinase L, R-Type Pyruvate Kinase, L Type Pyruvate Kinase, M Type Pyruvate Kinase, M1 Type Pyruvate Kinase, M2 Type Pyruvate Kinase, Pyruvate Kinase, L-Type
Pyruvate Metabolism, Inborn Errors - 새창 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.
Synonyms :
Pyruvate Oxidase - 새창
Synonyms : Oxidase, Pyruvate