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

MeSH(Medical Subject Headings) 맞춤 검색 (http://www.nlm.nih.gov) 결과 : 4 페이지: 1

Pyruvate Dehydrogenase (Lipoamide) - 새창 The E1 component of the multienzyme PYRUVATE DEHYDROGENASE COMPLEX. It is composed of 2 alpha subunits (pyruvate dehydrogenase E1 alpha subunit) and 2 beta subunits (pyruvate dehydrogenase E1 beta subunit).
Synonyms : Pyruvate Decarboxylase (Lipoamide), Pyruvate Dehydrogenase Component E1, Pyruvate Dehydrogenase-E1, Pyruvate Dehydrogenase E1
Pyruvate Dehydrogenase (Lipoamide)-Phosphatase - 새창 (Pyruvate dehydrogenase (lipoamide))-phosphate phosphohydrolase. A mitochondrial enzyme that catalyzes the hydrolytic removal of a phosphate on a specific seryl hydroxyl group of pyruvate dehydrogenase, reactivating the enzyme complex. EC 3.1.3.43.
Synonyms : Pyruvate Dehydrogenase Phosphate Phosphatase, Phosphatase, PDH, Phosphatase, Pyruvate Dehydrogenase
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