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Enzymes & cofactors

Search by name, gene symbol, EC number or the disorder it causes. Open any enzyme to see every reaction it catalyses, what accelerates it and what inhibits it.

Enzymes Cofactor terms Vitamins & minerals
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EC 2.1.3.3 OTC 1 reactions

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

EC 1.14.16.1 PAH 1 reactions

Phenylketonuria: musty odour, intellectual disability, hypopigmentation; BH4 defects cause malignant PKU