epitope corresponding to amino acids 627-926 mapping at the C-terminus of AASS of human origin
recommended for detection of AASS of mouse, rat and human origin by WB, IP, IF and ELISA; also reactive with additional species, including equine, bovine and porcine
AASS Background Information Alpha-aminoadipic semialdehyde synthase (AASS), also designated lysine ketoglutarate reductase (LKR) or saccharopine dehydrogenase (SDH), is a 926 amino acid protein that exists as a homodimer in the mitochondria. AASS acts as a bifunctional enzyme containing the lysine alpha-ketoglutarate reductase (LKR) and saccharopine dehydrogenase activities that catalyzes the first two steps in lysine degradation. It is widely expressed with highest expression in liver and transcription of the AASS gene is induced upon starvation. Mutations in the gene encoding AASS result in various forms familial hyperlysinemias (FH), autosomal recessive disorders characterized by hyperlysinemia, lysinuria, and variable saccharopinuria. However, no adverse mental or physical effects have been found in patients with hyperlysinemia.
AASS (H-300)
Clicca sull'immagine per ingrandirla
AASS (H-300): sc-134661. Western blot analysis of AASS expression in non-transfected: sc-117752 (A) and mouse AASS transfected: sc-118147 (B) 293T whole cell lysates.
