Anti-AASS antibody [EPR9145(B)] - BSA and Azide free

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

The AASS also known as Aminoadipate-semialdehyde Synthase is a mitochondrial enzyme that plays a role in lysine degradation. It has alternative names like LKRSDH or Lysine Ketoglutarate Reductase/Saccharopine Dehydrogenase. AASS is a bifunctional enzyme that catalyzes two sequential reactions in lysine catabolism. The first reaction converts lysine into saccharopine and the second converts saccharopine to aminoadipate semialdehyde. The protein possesses an approximate mass of 125 kDa. The expression of AASS occurs mainly in liver and kidney tissues although it can be detected at lower levels in other organs.

Biological function summary

Enzymes like AASS are critical in the metabolism of amino acids particularly within the lysine degradation pathway. The AASS enzyme does not form a larger complex but its function is essential for preventing lysine accumulation in cells. When lysine gets degraded it assists in maintaining cellular homeostasis by producing metabolites necessary for energy production and other metabolic processes. AASS functions independently without forming catalytic subunits with other proteins.

Pathways

Lysine degradation involves AASS as a significant player. This pathway leads to the production of acetyl-CoA an essential molecule in the citric acid cycle and energy production. Another important pathway involving AASS is the production of glutaryl-CoA. During these pathways AASS-related proteins include other enzymes like saccharopine reductase and dehydrogenase which participate sequentially to manage amino acid breakdown.

Associated diseases and disorders

Abnormal AASS function is associated with hyperlysinemia and saccharopinuria. Hyperlysinemia results from disrupted lysine degradation leading to excess lysine in the blood. Researchers have noted that AASS mutations may impact lysine metabolism causing these conditions. In comparison saccharopinuria characterized by excess saccharopine in urine directly connects to inadequate AASS activity. These conditions highlight how changes in AASS function can cause metabolic imbalances leading to disease.