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Carbamoyl Phosphate Synthetase I (CPS1)
- Target
- CPS1
- Molecular classification
- Enzyme
01
Overview
Carbamoyl phosphate synthetase I (CPS1) is a mitochondrial enzyme that catalyzes the first committed step of the urea cycle, which is essential for detoxifying ammonia. Deficiency leads to hyperammonemia and neurological complications.
- Other names
- CPSICongenital hyperammonemia type ICarbamyl-phosphate synthetase I deficiency
02
Mechanism of action
Not applicable; CPS1 is a target, not a drug.
03
Biological functions
Urea cycleAmmonia detoxification
04
Disease associations
Carbamoyl-phosphate synthetase I deficiencyHyperammonemia
05
Safety considerations
HyperammonemiaNeurological impairmentComa
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