Defects in this pathway leads to HHH syndrome and lysinuric protein intolerance, and one intermediate in this pathway can be decarboxylated to putresciene.
Defects in this pathway leads to HHH syndrome and lysinuric protein intolerance, and one intermediate in this pathway can be decarboxylated to putresciene. The main enzyme of this process is controlled allosterically by N-acetylglutamate, and that enzyme, mitochondrial carbamoyl phosphate synthetase, transfers a group from glutamine to phosphorylated bicarbonate. It is linked to the Krebs cycle via the creation of fumarate, and ther intermediates in this process include citrulline and ornithine, the latter of which is produced by the cleavage of arginine. FTP, name this cycle that takes place in the cytosol and mitochondria, responsible for creating a namesake nitrogenous molecule and component of urine.
Defects in this pathway leads to HHH syndrome and lysinuric protein intolerance, and one intermediate in this pathway can be decarboxylated to putresciene.
Urea cycle