Search Thermo Fisher Scientific
Search Thermo Fisher Scientific
应用 | 建议稀释比 | 已发表文章 |
---|---|---|
免疫印迹 (WB) |
1:200-1:1,000 | - |
免疫组化(石蜡) (IHC (P)) |
1:5-1:20 | - |
产品规格 | |
---|---|
种属反应 |
Human |
宿主/亚型 |
Rabbit / IgG |
分类 |
Polyclonal |
类型 |
Antibody |
抗原 |
Synthetic peptide corresponding to a region derived from internal residues of human GTP cyclohydrolase 1 |
偶联物 |
Unconjugated |
形式 |
Liquid |
浓度 |
0.5 mg/mL |
纯化类型 |
Antigen affinity chromatography |
保存液 |
PBS, pH 7.4, with 40% glycerol |
内含物 |
0.05% sodium azide |
保存条件 |
-20°C |
RRID |
AB_2635961 |
The antibody detects endogenous levels of total GCH1 protein.
This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin 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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: dystonia 14; GTP cyclohydrolase 1; GTP cyclohydrolase I; GTP-CH-I; guanosine 5'-triphosphate cyclohydrolase I
基因别名: DYT14; DYT5; DYT5a; GCH; GCH1; GTP-CH-1; GTPCH1; HPABH4B
UniProt ID: (Human) P30793
Entrez Gene ID: (Human) 2643