{"symbol":"AASS","synonyms":["LORSDH","LKR/SDH","LKRSDH"],"name":"aminoadipate-semialdehyde synthase","description":"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. [provided by RefSeq, Jul 2008]","ncbiEntrezGeneId":10157,"ncbiEntrezGeneUrl":"http://www.ncbi.nlm.nih.gov/gene/10157","proteins":[{"symbol":"AASS_HUMAN","href":"/api/1.0/protein/AASS_HUMAN"}],"hgncRootFamilies":[]}