Protein Name ornithine transcarbamylase
Organism Homo sapiens
Gene ID 5009
Gene Symbol

OTC

UniProt P00480 (OTC_HUMAN)
Relationships Total Number of functionally related compound(s) : 122
Total Number of Articles : 293
Description

ornithine transcarbamylase

Gene Summary

This nuclear gene encodes a mitochondrial matrix enzyme. The encoded protein is involved in the urea cycle which functions to detoxify ammonia into urea for excretion. Mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. [provided by RefSeq, May 2022]

synonyms
  • ornithine transcarbamylase, mitochondrial
  • ornithine carbamoyltransferase, mitochondrial
  • OCTD
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Properties