| saccharopine | HOOC(CH2)2CH(COOH)NH(CH2)4CH(NH2)COOH;a derivative of alpha-ketoglutarate and l-lysine that is an intermediate in l-lysine catabolism; elevated in cases of saccharopinuria. (05 Mar 2000) |
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| saccharopine dehydrogenase | Two enzymes that are used in the pathway of l-lysine catabolism; the first isoform catalyses the reversible conversion of l-lysine, alpha-ketoglutarate, and NADH to saccharopine and NAD+; the other isoform reversibly catalyses to conversion of saccharopine and NAD+ to l-glutamate, NADH, and l-alpha-aminoadipate d-saemialdehyde. A deficiency of one of these isoforms is associated with familial hyperlysinaemia and saccharopinuria. (05 Mar 2000) |
| saccharopine dehydrogenases | <enzyme> N-5-(1,3-dicarboxypropyl)-l-lysine:NAD(p)+ oxidoreductase (l-lysine-forming). Catalyses the oxidative cleavage of saccharopine to lysine plus ketoglutaric acid. Requires NAD; EC 1.5.1.8 requires NADP. Registry number: EC 1.5.1. (12 Dec 1998) |
| saccharopine oxidase | <enzyme> Flavoenzyme which catalyses the oxidative cleavage of saccharopine to delta-1-piper9deine-6-carboxylate, glutamate and h2o2 Registry number: EC 1.5.3.- (26 Jun 1999) |
Synonyms : Lysine-2-Oxoglutarate Reductase, Lysine-Ketoglutarate Reductase, Saccharopine Dehydrogenase (NAD+, L-Glutamate Forming), Saccharopine Dehydrogenase (NAD+, L-Lysine Forming), Saccharopine Dehydrogenase (NADP+, L-Glutamate Forming)
| saccharopine |
an intermediate in the metabolism of lysine, formed by condensation of lysine and α-ketoglutarate; it accumulates abnormally in some disorders of lysine degradation.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| saccharopine dehydrogenase (NAD+, L-glutamate-forming) |
[EC 1.5.1.9] an enzyme activity that catalyzes oxidative cleavage of saccharopine to form α-aminoadipate semialdehyde and glutamate, using NAD+ as an electron acceptor. The reaction is the second 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 the variant saccharopinuria.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| saccharopine dehydrogenase (NADP+, L-lysine-forming) |
[EC 1.5.1.8] 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. Usually called lysine ketoglutarate reductase.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| saccharopinemia |
an excess of saccharopine in the blood, as in hyperlysinemia or saccharopinuria.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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