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AASS is a bifunctional enzyme that catalyzes the first two steps in the mammalian lysine degradation pathway. The N-terminal and the C-terminal portions of this enzyme contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively, resulting in the conversion of lysine to alpha-aminoadipic semialdehyde. Mutations in the gene encoding this protein are associated with familial hyperlysinemia.
AASS; aass {ECO:0000312; alpha-aminoadipate semialdehyde synthase; alpha-aminoadipic semialdehyde synthase, mitochondrial; aminoadipate-semialdehyde synthase; aminoadipic semialdehyde synthase; EMBL:AAI28772.1}; LKR; LKR/SDH; LKRSDH; LOR; LOR/SDH; Lorsdh; Lysine ketoglutarate reductase; lysine oxoglutarate reductase; lysine-2-oxoglutarate reductase; lysine-ketoglutarate reductase /saccharopine dehydrogenase; saccharopine dehydrogenase; SDH
100 µL
100 µg
100 µL
100 µL
100 µL
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