Urea Cycle Disorder

Urea Cycle Disorder, also known as disorders of metabolism of ornithine, citrulline, argininosuccinic acid, arginine and ammonia, is related to carbamoyl phosphate synthetase i deficiency, hyperammonemia due to and n-acetylglutamate synthase deficiency. An important gene associated with Urea Cycle Disorder is ASL (Argininosuccinate Lyase), and among its related pathways/superpathways are Metabolism and Regulation of expression of SLITs and ROBOs. The drugs Phenylbutyric acid and Glycerin have been mentioned in the context of this disorder. Affiliated tissues include liver and brain, and related phenotypes are homeostasis/metabolism and renal/urinary system