您的位置：首页 > 产品中心 > Monoclonal Anti-GCH1 antibody produced in mouse
Monoclonal Anti-GCH1 antibody produced in mouse
| 产品编号： | 3145503 |
| 规格： | clone 2C4, purified immunoglobulin, buffered aqueous solution |
| 包装规格： | 100 μG |
| 产品类别： | 进口试剂 |
| 品牌： | Sigma-Aldrich |
| 优惠价： | 立即咨询 |
产品别名
Monoclonal Anti-GCH1 antibody produced in mouse
DYT5
GTP-CH-1
GTPCH1
DYT14
GCH
基本信息
| NACRES | NA.41 |
| General description【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) |
| Immunogen【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 |
| Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
产品性质
| Quality Level【质量水平】 | 100 |
| biological source【生物来源】 | mouse |
| conjugate【偶联物】 | unconjugated |
| antibody form【抗体形式】 | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone【克隆】 | 2C4, monoclonal |
| form【形式】 | buffered aqueous solution |
| mol wt【分子量】 | antigen ~53.61 kDa |
| species reactivity | human |
| technique(s) | capture ELISA: suitable indirect ELISA: suitable indirect immunofluorescence: suitable |
| isotype【同位素/亚型】 | IgG1κ |
| NCBI accession no.【NCBI登记号】 | BC025415 |
| UniProt accession no.【UniProt登记号】 | P30793 |
| shipped in【运输】 | dry ice |
| storage temp.【储存温度】 | −20℃ |
| Gene Information | human ... GCH1(2643) |
产品说明
| Disclaimer【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. |
安全信息
| Storage Class Code【储存分类代码】 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
