Search results

...specifically used for hyperammonemia due to [[N-acetylglutamate synthase]] deficiency and certain other [[urea cycle disorders]]. * [[N-acetylglutamate synthase]]: An enzyme that plays a crucial role in the urea cycle.

There are six main types of urea cycle disorders, each caused by a deficiency in one of the six enzymes involved in the urea cycle. These include: * [[Carbamoyl phosphate synthetase I deficiency]] ({{IPA|kɑːrˈbæmoɪl ˈfɑːsfeɪt sɪnˈθiːtɪs aɪ dɪˈfɪʃənsi

...dɪsˈɔːrdər}}) is a genetic disorder caused by a mutation that results in a deficiency of one of the six enzymes in the [[urea cycle]]. These enzymes are responsi ...the [[mitochondria]] and cytosol of the liver. The cycle is regulated by [[N-acetylglutamate]], which ensures that the rate of urea production is adequate to the body's