urea cycle diseases
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urea-cycle disorders, urea cycle disorder
Urea-cycle disorders (UCDs) are a group of inborn errors of hepatocyte metabolism that are caused by the loss of enzymes involved in the process of transferring nitrogen from ammonia to urea, via the urea cycle (UC).
Types
ornithine transcarbamylase deficiency
Features
References
Mian A, Lee B. Urea-cycle disorders as a paradigm for inborn errors of hepatocyte metabolism. Trends Mol Med. 2002 Dec;8(12):583-9. PMID: 12470992