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Monoclonal Anti-GCH1 antibody produced in mouse
| 产品编号： | 3145502 |
| 规格： | clone 4A12, 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
Anti-GCH
Anti-GTP cyclohydrolase 1 (dopa-responsive dystonia)
Anti-GTPCH1
Anti-DYT5
基本信息
| NACRES | NA.41 |
| General description【一般描述】 | Guanosine triphosphate (GTP) cyclohydrolase I (GCH1), a homodecameric protein belongs to the GTP cyclohydrolase family. Many isoforms of GCH1 are present due to alternative splicing. However, not all variants encode a functional enzyme. The GCH1 gene is mapped to human chromosome 14q22.2. |
| Immunogen【免疫原】 | GCH1 (NP_000152, 84 a.a. ~ 172 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence ENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIV |
| Biochem/physiol Actions【生化/生理作用】 | Guanosine triphosphate (GTP) cyclohydrolase I (GCH1) catalyzes the synthesis of 7,8-dihydroneopterin triphosphate from GTP. It acts as a rate-limiting enzyme in the tetrahydrobiopterin (BH4) biosynthesis pathway. GCH1 requires zinc (Zn2+) for its catalytic function. It is crucial for dopamine synthesis, and its variants may have a role in the pathogenesis of Parkinson′s disease (PD). Mutations in the GCH1 gene are implicated in Segawa disease, hyperphenylalaninemia, and dopa-responsive dystonia (DRD). GCH1 gene polymorphisms are correlated to the pathophysiology of obstructive sleep apnea (OSA) and in neuropathic pain and stroke. |
| Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
| Legal Information【法律信息】 | GenBank is a registered trademark of United States Department of Health and Human Services |
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】 | 4A12, monoclonal |
| form【形式】 | buffered aqueous solution |
| species reactivity | human |
| technique(s) | immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable immunoprecipitation (IP): suitable indirect ELISA: suitable western blot: 1-5 μg/mL |
| isotype【同位素/亚型】 | IgG2aκ |
| GenBank® accession no. | NM_000161 |
| 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】 | 12 - Non Combustible Liquids |
| Personal Protective Equipment【个人防护装备】 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |
