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Rabbit Anti-GCH1  antibody (bs-25514R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-25514R
英文名称 GCH1
中文名称 三磷酸鸟苷环水解酶抗体
别    名 GTP-CH-1; DYT 5; DYT5; GCH 1; GCH; GTP CH 1; GTP CH I; GTP cyclohydrolase 1 (dopa responsive dystonia); GTP cyclohydrolase 1; GTP cyclohydrolase I; GTPCH 1; GTPCH1; Guanosine 5' triphosphate cyclohydrolase I; DYT14; DYT5a; GCH1_HUMAN; HPABH4B.  
研究领域 免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human (predicted: Sheep,Horse,Cow,Dog,Chicken,Rat,Mouse)
产品应用 WB=1:500-2000, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 28kDa
细胞定位 细胞核 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GCH1: 101-200/250 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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, Jul 2008]

SWISS:
P30793

Gene ID:
2643

Database links:

Entrez Gene: 2643 Human

Entrez Gene: 14528 Mouse

Entrez Gene: 100286831 Rabbit

Entrez Gene: 29244 Rat

Omim: 600225 Human

SwissProt: P30793 Human

SwissProt: Q05915 Mouse

SwissProt: P51596 Rabbit

SwissProt: P22288 Rat

Unigene: 624900 Human

Unigene: 86724 Human

Unigene: 10651 Mouse

Unigene: 28195 Rat



产品图片
Sample:
Lane 1: Recombinant human GCH1 protein, C-His(bs-42069P)
Primary: Anti-GCH1 (bs-25514R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 28 kDa
Observed band size: 30 kDa
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