AASS Antibody / LKR / SDH

Alpha-aminoadipic semialdehyde synthase (AASS), also called Lysine ketoglutarate reductase (LKR) and Saccharopine dehydrogenase (SDH) is an enzyme encoded by the AASS gene in humans and is involved in their major lysine degradation pathway. This gene encodes 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 this gene are associated with familial hyperlysinemia.