6-Pyruvoyl tetrahydrobiopterin synthase catalyses the conversion of dihydroneopterin triphosphate to 6-pyruvoyl tetrahydropterin, the second of three enzymatic steps in the synthesis of tetrahydrobiopterin from GTP. The functional enzyme is a hexamer ...
6-Pyruvoyl tetrahydrobiopterin synthase catalyses the conversion of dihydroneopterin triphosphate to 6-pyruvoyl tetrahydropterin, the second of three enzymatic steps in the synthesis of tetrahydrobiopterin from GTP. The functional enzyme is a hexamer of identical subunits [1].
The enzyme 6-pyruvoyl tetrahyropterin synthase catalyses the second of three steps in the de novo biosynthesis of tetrahydrobiopterin, the conversion of dihydroneopterin triphosphate to 6-pyruvoyl tetrahyropterin. Tetrahydrobiopterin is the natural cofactor for several enzymes such as phenylalanine hydroxylase, tyrosine hydroxylase and tryptophan hydroxylase, nitric oxide synthase and glycerol ether monooxygenase. Lack of tetrahydrobiopterin leads to hyperphenylalaninaemia and a deficiency of the biogenic amine neurotransmitters, dopamine and serotonin and is accompanied by severe progressive mental retardation.
Defined by 7 residues: HIS:A-19 [auth A-23]CYS:A-38 [auth A-42]HIS:A-44 [auth A-48]HIS:A-46 [auth A-50]GLU:A-129 [auth A-133]ASP:B_6-84 [auth B_6-88]HIS:B_6-85 [auth B_6-89]
