产品概述：Recombinant protein of human GTP cyclohydrolase 1 (GCH1), full length, with N-terminal HIS tag, expressed in E.Coli, 50ug

蛋白长度：A DNA sequence encoding human full-length GCH1

表达系统：E. coli

种属：Human

标签类型：N-His

分子量：27.7 kDa

通用名：GCH1

总结：This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a functional enzyme. [provided by RefSeq, Jul 2008]

形式：Liquid

浓度：>50 ug/mL as determined by microplate BCA method

纯度：> 80% as determined by SDS-PAGE and Coomassie blue staining

储存溶液：25mM Tris, pH8.0, 150 mM NaCl, 10% glycerol, 1 % Sarkosyl.

别名：DYT5; DYT5a; DYT14; GCH; GTP-CH-1; GTPCH1; HPABH4B