产品说明
一般描述
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)
免疫原
GCH1 (NP_000152.1, 1 a.a. ~ 250 a.a) full-length human protein.
Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS
外形
Solution in phosphate buffered saline, pH 7.4
基本信息
| NACRES | NA.41 |
产品性质
| 质量水平 | 100 |
| 生物来源 | mouse |
| 偶联物 | unconjugated |
| 抗体形式 | purified immunoglobulin |
| antibody product type | primary antibodies |
| 克隆 | polyclonal |
| 形式 | buffered aqueous solution |
| 分子量 | antigen ~27.9 kDa |
| species reactivity | human |
| technique(s) | indirect immunofluorescence: suitable western blot: 1 μg/mL |
| NCBI登记号 | NM_000161.2 |
| UniProt登记号 | P30793 |
| 运输 | dry ice |
| 储存温度 | −20℃ |
| Gene Information | human ... GCH1(2643) |
安全信息
| 储存分类代码 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
Sigma-Aldrich
m.cnreagent.com
