P00966

ASS1(argininosuccinate synthase 1) plays a crucial role in the urea cycle, catalyzing the second-to-last step in the arginine biosynthetic pathway. This enzyme is essential for the detoxification of ammonia in the liver, converting citrulline and aspartate into argininosuccinate, which is subsequently cleaved to produce arginine and fumarate. A deficiency in ASS1 can lead to citrullinemia (CTLN1), an autosomal recessive disorder characterized by severe vomiting spells and cognitive impairments, highlighting the importance of ASS1 in metabolic health. The tetrameric structure of ASS1, composed of identical subunits, is vital for enzymatic function, as this composition allows for proper substrate binding and catalytic activity, making ASS1 protein interactions critical for understanding related metabolic disorders.