Target intelligence / Profile preview

Ornithine Carbamoyltransferase (OTC)

Target
OTC
Molecular classification
Enzyme, Transferase
01

Overview

Ornithine carbamoyltransferase (OTC) is a mitochondrial enzyme crucial for the urea cycle, catalyzing the conversion of carbamoyl phosphate and ornithine to citrulline and phosphate. This process is essential for detoxifying ammonia produced during protein metabolism. Deficiency in OTC, caused by mutations in the OTC gene, leads to hyperammonemia and severe neurological complications. OTC is a trimeric protein, with its active site located at the interface between monomers. It is primarily expressed in the liver and intestine, with different roles in ureagenesis and amino acid homeostasis, respectively.

Other names
Ornithine transcarbamylase
02

Mechanism of action

N/A

03

Biological functions

Urea cycleAmmonia detoxificationCitrulline synthesis
04

Disease associations

Ornithine transcarbamylase deficiency (OTCD)Hyperammonemia
05

Safety considerations

HyperammonemiaNeurological damageLiver failure
06

Interacting drugs

Nitrogen scavenging agents
07

Biomarkers

Ammonia levelsOTC enzyme activityGenetic mutations in OTC gene

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