lysine-ketoglutarate reductase
- lysine-ketoglutarate reductase
- ly·sine-ke·to·glu·ta·rate re·duc·tase (liґsēn ke″to-glooґtə-rāt re-dukґtās) an enzyme activity that catalyzes the condensation of L-lysine and α-ketoglutarate to form saccharopine, using NADPH as an electron donor. The reaction is the initial step in the major route of lysine degradation; the enzyme activity is part of the bifunctional enzyme α-aminoadipic semialdehyde synthase (q.v.). The enzyme activity is absent in hyperlysinemia and substantially reduced in the variant saccharopinuria. In official nomenclature called saccharopine dehydrogenase (NADP+, L-lysine-forming).
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Saccharopine dehydrogenase (NADP+, L-lysine-forming) — In enzymology, a saccharopine dehydrogenase (NADP+, L lysine forming) (EC number|1.5.1.8) is an enzyme that catalyzes the chemical reaction:N6 (L 1,3 dicarboxypropyl) L lysine + NADP+ + H2O ightleftharpoons L lysine + 2 oxoglutarate + NADPH +… … Wikipedia
Lysine — Lysine … Wikipedia
saccharopine dehydrogenase (NADP+ L-lysine-forming) — sac·cha·ro·pine de·hy·dro·gen·ase (NADP+, L ly·sine form·ing) (sakґə ro pēn″ de hiґdro jən ās liґsēn formґing) [EC 1.5.1.8] official nomenclature for lysine ketoglutarate reductase … Medical dictionary
hyperlysinemia — A metabolic disorder characterized by mental retardation, convulsions, anemia, and asthenia; associated with an abnormal increase of the amino acid lysine in the circulating blood due to a deficiency … Medical dictionary
AASS — Aminoadipate semialdehyde synthase, also known as AASS, is a human gene.cite web | title = Entrez Gene: AASS aminoadipate semialdehyde synthase| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView TermToSearch=10157|… … Wikipedia
saccharopinuria — Elevated levels of saccharopine in the urine; associated with a variant of familial hyperlysinuria. * * * sac·cha·ro·pin·uria (sak″ə ro pĭ nuґre ə) 1. excretion of saccharopine in the urine. 2. a variant form of… … Medical dictionary
α-aminoadipic semialdehyde synthase — α ami·no·adip·ic semi·al·de·hyde syn·thase (ə me″no ə dipґik sem″e alґdə hīd sinґthās) a bifunctional enzyme comprising the two enzyme activities lysine ketoglutarate reductase (q.v.) and saccharopine… … Medical dictionary
aminoadipic δ-semialdehyde synthase — A bifunctional enzyme used in lysine degradation; it has a lysine:α ketoglutarate reductase activity as well as a saccharopine dehydrogenase activity. A deficiency of this enzyme results in f … Medical dictionary
List of diseases (L) — A list of diseases in the English wikipedia.DiseasesTOC LaLab Lam* Labrador lung * Labyrinthitis * Lachiewicz Sibley syndrome * Lacrimo auriculo dento digital syndrome * Lactate dehydrogenase deficiency type A * Lactate dehydrogenase deficiency… … Wikipedia
Amino acid — This article is about the class of chemicals. For the structures and properties of the standard proteinogenic amino acids, see Proteinogenic amino acid. The generic structure of an alpha amino acid in its unionized form … Wikipedia
