基因名:
GCH1
产品别名:
DYT14; DYT5; DYT5a; GCH; GTP-CH-1; GTPCH1; HPABH4B; GCH1; GTP cyclohydrolase 1; GTP cyclohydrolase 1; GTP cyclohydrolase 1; GTP cyclohydrolase I; GTP-CH-I; dystonia 14; guanosine 5'-triphosphate cyclohydrolase I; GTP环化水解酶1(GCH1); 三磷酸鸟苷环水解酶;
背景信息:
GTP cyclohydrolase I (also designated dopa-responsive dystonia) catalyzes the conversion of GTP to D-erythro-7,8-dihydroneopterin triphosphate, the first and rate-limiting step in tetrahydrobiopterin (BH4) biosynthesis. Tetrahydrobiopterin is an essential cofactor for 3 aromatic amino acid monooxygenases: phenylalanine, tyrosine, and tryptophan hydroxylases. Animals can synthesize tetrahydrobiopterin in vivo from GTP through several enzymatic reactions.