AAI40944.1
[Other Products]
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
116,038 Da
NCBI Official Full Name
Carbamoyl-phosphate synthetase 1, mitochondrial
NCBI Official Synonym Full Names
carbamoyl-phosphate synthase 1
NCBI Official Synonym Symbols
NCBI Protein Information
carbamoyl-phosphate synthase [ammonia], mitochondrial
UniProt Protein Name
Carbamoyl-phosphate synthase [ammonia], mitochondrial
UniProt Synonym Protein Names
Carbamoyl-phosphate synthetase I; CPSase I
UniProt Synonym Gene Names
NCBI Summary for CPS1
The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
UniProt Comments for CPS1
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Research Articles on CPS1
Precautions
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Products associated with CPS1 elisa kit
Pathways associated with CPS1 elisa kit
Organs/Tissues associated with CPS1 elisa kit
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