Editing Urea cycle (section)

=== Individual disorders ===
* [[N-Acetylglutamate synthase deficiency|N-Acetylglutamate synthase (NAGS) deficiency]]
* [[Carbamoyl phosphate synthetase I deficiency|Carbamoyl phosphate synthetase (CPS) deficiency]]
* [[Ornithine transcarbamoylase deficiency|Ornithine transcarbamoylase (OTC) deficiency]]
*  [[Citrullinemia|Citrullinemia type I]] (Deficiency of argininosuccinic acid synthase)
* [[Argininosuccinic aciduria]] (Deficiency of argininosuccinic acid lyase)
* [[Argininemia]] (Deficiency of arginase)
* [[Hyperornithinemia, hyperammonemia, homocitrullinuria syndrome|Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome]] (Deficiency of the mitochondrial ornithine transporter)<ref name=":4" /><ref>{{Citation|last1=Smith|first1=L. D.|title=Chapter 5: Urea cycle and other disorders of hyperammonemia|date=2017-01-01|url=http://www.sciencedirect.com/science/article/pii/B9780128028964000043|work=Biomarkers in Inborn Errors of Metabolism|pages=103–123|editor-last=Garg|editor-first=Uttam|place=San Diego|publisher=Elsevier|language=en|doi=10.1016/b978-0-12-802896-4.00004-3|isbn=978-0-12-802896-4|access-date=2020-11-10|last2=Garg|first2=U.|editor2-last=Smith|editor2-first=Laurie D.}}</ref>

All urea cycle defects, except OTC deficiency, are inherited in an [[autosomal recessive]] manner. OTC deficiency is inherited as an [[X-linked recessive]] disorder, although some females can show symptoms. Most urea cycle disorders are associated with [[hyperammonemia]], however argininemia and some forms of argininosuccinic aciduria do not present with elevated ammonia.