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Anti-GCH1 antibody produced in mouse
| 产品编号： | 3364344 |
| 规格： | purified immunoglobulin, buffered aqueous solution |
| 包装规格： | 50 μG |
| 产品类别： | 进口试剂 |
| 品牌： | Sigma-Aldrich |
| 优惠价： | 立即咨询 |
产品别名
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 (NP_000152.1, 1 a.a. ~ 250 a.a) full-length human protein. 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】 | polyclonal |
| form【形式】 | buffered aqueous solution |
| mol wt【分子量】 | antigen ~27.9 kDa |
| species reactivity | human |
| technique(s) | indirect immunofluorescence: suitable western blot: 1 μg/mL |
| NCBI accession no.【NCBI登记号】 | NM_000161.2 |
| 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) |
安全信息
| Storage Class Code【储存分类代码】 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
