



AASS Antibody (D-8) is a mouse monoclonal IgG2a antibody that detects alpha-aminoadipic semialdehyde synthase (AASS) in mouse, rat, and human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry, and enzyme-linked immunosorbent assay (ELISA). AASS is a 926 amino acid protein that functions as a homodimer located in the mitochondria, where AASS plays a crucial role in lysine degradation by catalyzing the first two steps of this metabolic pathway through bifunctional enzyme activities: lysine alpha-ketoglutarate reductase and saccharopine dehydrogenase. AASS regulates lysine levels through widespread expression, particularly in the liver, and AASS gene transcription increases during starvation, highlighting a role in metabolic adaptation. Mutations in the AASS gene can lead to familial hyperlysinemias, which are autosomal recessive disorders characterized by elevated lysine levels, lysinuria, and variable saccharopinuria; however, patients with hyperlysinemia typically do not experience adverse mental or physical effects. Anti-AASS antibody (D-8) serves as an essential tool for researchers studying lysine metabolism and associated disorders, providing reliable detection and analysis of this critical protein.
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
AASS Antibody (D-8) | sc-374322 | 200 µg/ml | $322.00 | |||
AASS Antibody (D-8): m-IgG Fc BP-HRP Bundle | sc-540436 | 200 µg Ab; 10 µg BP | $361.00 |