Carbamoyl phosphate synthetase (CPS)- l and II

Feature

CPS-l

CPS-ll

Full Name

Carbamoyl Phosphate
Synthetase I

Carbamoyl Phosphate
Synthetase II

Location

Mitochondria

Cytosol

Pathway

Urea Cycle

Pyrimidine Synthesis

Substrate

Ammonia (NH3)

Glutamine (Gln)

Activator

N-Acetylglutamate (NAG)

PRPP (Phosphoribosyl
pyrophosphate) and ATP

Function

Converts ammonia and bicarbonate
into carbamoyl phosphate

Converts glutamine and bicarbonate
into carbamoyl phosphate

Expression

Primarily in liver

Found in many tissues

Clinical
Relevance

Deficiency can lead to
hyperammonemia type-1

Important for DNA and
RNA synthesis