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FIGURE: 1 / 1
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.
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Protein Aliases: aass {ECO:0000312|EMBL:AAI28772.1}; alpha-aminoadipate semialdehyde synthase; Alpha-aminoadipic semialdehyde synthase, mitochondrial; aminoadipic semialdehyde synthase; LKR/SDH; lysine oxoglutarate reductase; lysine-2-oxoglutarate reductase; lysine-ketoglutarate reductase /saccharopine dehydrogenase; saccharopine dehydrogenase
Gene Aliases: AASS; LKR; LKR/SDH; LKRSDH; LOR; LOR/SDH; LORSDH; SDH
UniProt ID: (Human) Q9UDR5, (Mouse) Q99K67, (Rat) A2VCW9
Entrez Gene ID: (Human) 10157, (Mouse) 30956, (Rat) 296925
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