CPS1 Antibody from BIOLEGEND

CPS1, or carbamoyl phosphate synthase 1, catalyzes the initial step in the urea cycle to produce carbamoyl phosphate from ammonia and bicarbonate. CPS1 is highly expressed in the mitochondria of hepatocytes, where a robust urea cycle takes place to detoxify ammonia from protein catabolism. CPS1 deficiency in humans is an autosomal recessive disease that causes severe symptoms, such as hyperammonemia, in urea cycle disorders. This deficiency affects the neuronal and metabolic development of newborn children. CPS1 undergoes various post-translational modifications, including acetylation, succinylation, and glutarylation, which regulate the enzymatic activity of CPS1. During fasting, sirtuin 5 (SIRT5) is activated by increased NAD levels, leading to the deacetylation and deglutarylation of CPS1. This process removes inhibitory modifications from the active ATP binding site. The tumor suppressor p53 regulates ammonia metabolism by repressing the urea cycle and the expression of genes such as CPS1, OTC, and ARG1. The repression leads to the inhibition of ureagenesis and the elimination of ammonia, ultimately impacting tumor growth.