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Monoclonal Anti-GCH1 antibody produced in mouse

品牌
Sigma-Aldrich
货号
WH0002643M1
规格纯度
clone 4A12, purified immunoglobulin, buffered aqueous solution
参考价格
4888.5 *本价格含增值税费
促销
服务
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数量
-+
产品名称:
Monoclonal Anti-GCH1 antibody produced in mouse
Anti-GCH
Anti-GTP cyclohydrolase 1 (dopa-responsive dystonia)
Anti-GTPCH1
Anti-DYT5
产品介绍:

产品说明

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.

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

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.

外形

Solution in phosphate buffered saline, pH 7.4

法律信息

GenBank is a registered trademark of United States Department of Health and Human Services

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

NACRESNA.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 typeprimary antibodies
克隆4A12, monoclonal
形式buffered aqueous solution
species reactivityhuman
technique(s)immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
immunoprecipitation (IP): suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL
同位素/亚型IgG2aκ
GenBank® accession no.NM_000161
UniProt登记号P30793
运输dry ice
储存温度−20℃
Gene Informationhuman ... GCH1(2643)

安全信息

储存分类代码12 - Non Combustible Liquids
闪点(F)Not applicable
闪点(C)Not applicable
个人防护装备Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

Sigma-Aldrich

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