5o1p
Jump to navigation
Jump to search
Crystal structure of human aminoadipate semialdehyde synthase, saccharopine dehydrogenase.Crystal structure of human aminoadipate semialdehyde synthase, saccharopine dehydrogenase.
Structural highlights
DiseaseAASS_HUMAN Hyperlysinemia;Saccharopinuria. The disease is caused by mutations affecting the gene represented in this entry. The protein represented in this entry is involved in disease pathogenesis. A selective decrease in mitochondrial NADP(H) levels due to NADK2 mutations causes a deficiency of NADPH-dependent mitochondrial enzymes, such as DECR1 and AASS.[1] FunctionAASS_HUMAN Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively. References
|
|