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MS
unreviewed

Ornithine transcarbamylase (OTC)

1 reactions · 1 pathways

Cofactors used

Clinical / pharmacological. OTC deficiency (X-linked, commonest urea cycle defect): hyperammonaemia with HIGH orotic acid, low BUN

What it does, reaction by reaction

1 reactions

Urea Cycle Amino Acid & Nitrogen Metabolism · Mitochondrial matrix + cytosol

step 2 Irreversible

Carbamoyl phosphate + ornithine → citrulline + Pᵢ

Converts Carbamoyl phosphate Ornithine into Citrulline Pi

Notes

Ornithine transcarbamylase (OTC) catalyzes this mitochondrial reaction without direct ATP use. Citrulline is exported to the cytosol in exchange for ornithine; the reaction is effectively irreversible under physiological conditions because carbamoyl phosphate is high energy.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

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