CTPS1


Description

The CTPS1 (CTP synthase 1) is a protein-coding gene located on chromosome 1.

CTP synthase 1 (CTPS1) is an enzyme encoded by the CTPS1 gene in humans. It is involved in the de novo pyrimidine synthesis pathway and catalyzes the conversion of uridine triphosphate (UTP) to cytidine triphosphate (CTP). CTP is a crucial building block for the production of DNA, RNA, and some phospholipids. CTPS1 is an asymmetrical homotetramer, with only three of its four monomers contributing to the catalytic domain. It requires adenosine triphosphate (ATP), UTP, and the amino acid glutamine for enzymatic activity. The ATP and UTP binding domains are located at the tetramer interface, while the glutamine binding domain is located away from the interface. Glutamine is hydrolyzed by the glutamine amidotransferase domain on the outside of the CTPS1 enzyme, and the ammonia produced is channeled through to the synthase domain in the interior of the enzyme, to the tetrameric interface. ATP-dependent phosphorylation of UTP produces 4-phosphoryl UTP, which reacts with the ammonia to produce CTP. The reaction can also occur using ammonia in solution instead of the glutamine-derived ammonia. Guanosine triphosphate (GTP) is an allosteric activator of enzyme activity, stimulating the hydrolysis of glutamine.

CTPS1 is also known as CTPS, GATD5, GATD5A, IMD24.

Associated Diseases


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