The CPS1 protein, also known as carbamoyl phosphate synthetase I, plays a crucial role in the urea cycle. The CPS1 protein has a molecular weight of 163kDa and is primarily located in the mitochondria of the liver. It is a key enzyme in the urea cycle, responsible for synthesizing urea from ammonia and CO2 in the body. This process is essential for eliminating excess ammonia in the body, making it crucial for ammonia detoxification in animals. Mutations in the CPS1 can lead to carbamoyl phosphate synthetase I deficiency (CPSID), a rare autosomal recessive genetic disorder that causes abnormalities in ammonia metabolism. This condition triggers a genetic metabolic disease characterized by hyperammonemia. Studies have shown that the CPS1 is closely related to various malignancies such as liver cancer, lung cancer, and colorectal cancer. It may affect the occurrence and development of tumors through multiple signal transduction pathways or by influencing pyrimidine synthesis.