Defects in this pathway such as OTCD can be treated with drugs such as Buphenyl.
Defects in this pathway such as OTCD can be treated with drugs such as Buphenyl. One enzyme in this pathway, CPS-I, is allosterically regulated by N-acetylglutamate. In the first step, one of the products reacts with carbamoyl phosphate to produce citrulline. In another step, argininosuccinate lyase produces a molecule that is reconverted into (*) aspartate and a molecule which is cleaved in the next step, fumarate and arginine. Each turn of it inputs one bicarbonate ion and 3 ATP and outputs one water molecule, one AMP, and 2 ADP. Also known as the Krebs-Henseleit or ornithine cycle, for 10 points, name this biological pathway taking place in the liver where ammonia is converted into the namesake product to be excreted in urine.
Defects in this pathway such as OTCD can be treated with drugs such as Buphenyl.
urea cycle [accept Krebs-Henseleit or ornithine cycle before mention, do NOT accept or prompt Krebs cycle]