Alpha-aminoadipic semialdehyde synthase, mitochondrial

Alpha-aminoadipic semialdehyde synthase, mitochondrial is an is_associated_with::enzyme that in humans is encoded by the AASS is_associated_with::gene.

Function
This gene encodes a is_associated_with::bifunctional enzyme that catalyzes the first two steps in the mammalian is_associated_with::lysine degradation pathway. The is_associated_with::N-terminal and the is_associated_with::C-terminal portions of this enzyme contain is_associated_with::lysine-is_associated_with::ketoglutarate is_associated_with::reductase and is_associated_with::saccharopine dehydrogenase activity, respectively, resulting in the conversion of lysine to alpha-aminoadipic semialdehyde. Mutations in this gene are associated with familial is_associated_with::hyperlysinemia.