| 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.
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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.
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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.
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| saccharopinemia |
an excess of saccharopine in the blood, as in hyperlysinemia or saccharopinuria.
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| saccharopinuria |
1. excretion of saccharopine in the urine. 2. a variant form of hyperlysinemia due to partial deficiency of α-aminoadipic semialdehyde synthase activity; the activity of saccharopine dehydrogenase (NAD+, L-glutamate-forming) is absent, but substantial saccharopine dehydrogenase (NADP+, L-lysine-forming) activity is retained. It is clinically similar to hyperlysinemia but with higher urinary saccharopine and lower lysine.
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