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 familial hyperlysinemia.

Medical dictionary. 2011.

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  • 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

  • AASS — Aminoadipate semialdehyde synthase, also known as AASS, is a human gene.cite web | title = Entrez Gene: AASS aminoadipate semialdehyde synthase| url = Cmd=ShowDetailView TermToSearch=10157|… …   Wikipedia

  • Saccharopine dehydrogenase (NAD+, L-glutamate-forming) — In enzymology, a saccharopine dehydrogenase (NAD+, L glutamate forming) (EC number| is an enzyme that catalyzes the chemical reaction:N6 (L 1,3 dicarboxypropyl) L lysine + NAD+ + H2O ightleftharpoons L glutamate + 2 aminoadipate 6… …   Wikipedia

  • 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

  • saccharopine dehydrogenase — sac·cha·ro·pine de·hy·dro·gen·ase (sakґə ro pēn″ de hiґdro jən ās) an enzyme activity that catalyzes oxidative cleavage of saccharopine to form α aminoadipate semialdehyde and glutamate, using NAD+ as an electron… …   Medical dictionary

  • Saccharopinuria — Infobox Disease Name = PAGENAME Caption = Saccharopine Width = 125px DiseasesDB = ICD10 = ICD9 = ICD9|270.7 ICDO = OMIM = 268700 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = Saccharopinuria (an excess of saccharopine in the urine),… …   Wikipedia

  • Hyperlysinemia — Infobox Disease Name = PAGENAME Caption = lysine DiseasesDB = 33215 ICD10 = ICD9 = ICD9|270.7 ICDO = OMIM = 238700 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = D020167 Hyperlysinemia is an autosomal recessive [cite journal… …   Wikipedia

  • hyperlipemia — Elevated levels of lipids in the blood plasma. There are several types of h.. One is associated with a deficiency of δ aminoadipic semialdehyde synthase. SEE ALSO: lipemia. carbohydrate induced h. SYN: type III familial hyperlipoproteinemia, type …   Medical dictionary

  • 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

  • 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.… …   Medical dictionary

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