Carbamoyl phosphate synthetase I

Carbamoyl phosphate synthetase I (pronunciation: kar-ba-moyl fos-fate sin-thet-ase one) is an enzyme that in humans is encoded by the CPS1 gene. It is involved in the urea cycle, which is a series of biochemical reactions in mammals during the production of urea from ammonia. This enzyme is found in the mitochondria of liver cells.

Etymology

The term "Carbamoyl phosphate synthetase I" is derived from its function. "Carbamoyl" refers to the carbamoyl group (-CONH2), "phosphate" refers to the phosphate group (-PO4), and "synthetase" refers to the type of enzyme that catalyzes the joining of two molecules. The "I" denotes that it is the first enzyme in the urea cycle.

Function

Carbamoyl phosphate synthetase I is the first enzyme in the urea cycle and plays a key role in removing excess ammonia from the body. It catalyzes the synthesis of carbamoyl phosphate from bicarbonate, ammonia, and adenosine triphosphate (ATP).

Related Terms

• Urea cycle
• Enzyme
• Gene
• Ammonia
• Urea
• Mitochondria
• Bicarbonate
• Adenosine triphosphate

See Also

• Carbamoyl phosphate synthetase II
• Ornithine transcarbamylase
• Argininosuccinate synthase
• Argininosuccinate lyase
• Arginase

External links

• Medical encyclopedia article on Carbamoyl phosphate synthetase I
• Wikipedia's article - Carbamoyl phosphate synthetase I

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