半岛bd体育手机客户端 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 (AAH25415.1, 1 a.a. ~ 250 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS
外形
Solution in phosphate buffered saline, pH 7.4
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
NACRES | NA.41 |
半岛bd体育手机客户端 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 | 2C4, monoclonal |
形式 | buffered aqueous solution |
分子量 | antigen ~53.61 kDa |
species reactivity | human |
technique(s) | capture ELISA: suitable indirect ELISA: suitable indirect immunofluorescence: suitable |
同位素/亚型 | IgG1κ |
NCBI登记号 | BC025415 |
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