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Rabbit Anti-GCH1  antibody (bs-25514R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-25514R
英文名稱 Rabbit Anti-GCH1  antibody
中文名稱 三磷酸鳥苷環(huán)水解酶抗體
別    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.  
研究領域 免疫學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human (predicted: Mouse,Rat,Sheep,Cow,Chicken,Dog,Horse)
產品應用 WB=1:500-2000,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 28kDa
細胞定位 細胞核 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GCH1: 101-200/250 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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



產品圖片
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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