Aminoadipate-semialdehyde synthase (AASS) is a mitochondrial enzyme made up of 927 amino acids. The gene encoding it has 24 exons and is localized to human chromosome 7q31.3.
Synonyms: Anti-Alpha-aminoadipic semialdehyde synthase, mitochondrial; Anti-LKR; Anti-LKR/SDH; Anti-LOR; Anti-SDH
Storage: -20C
Application: Anti-AASS antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem Physiol Actions: Aminoadipate-semialdehyde synthase (AASS) is involved in the catabolism of lysine. It converts lysine to saccharopine and this is further oxidised to form a-aminoadipic semialdehyde. Mutations in the AASS gene have been shown to be linked to hyperlysinemia.
Legal Information: Prestige Antibodies is a registered trademark of Sigma-Aldrich Co. LLC