| 產(chǎn)品編號(hào) | 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(lǐng)域 | 免疫學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human (predicted: Mouse,Rat,Sheep,Cow,Chicken,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 28kDa |
| 細(xì)胞定位 | 細(xì)胞核 細(xì)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(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)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 Omim: 600225 Human SwissProt: P30793 Human SwissProt: Q05915 Mouse SwissProt: P51596 Rabbit Unigene: 624900 Human Unigene: 86724 Human Unigene: 10651 Mouse Unigene: 28195 Rat |
| 產(chǎn)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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