| 克隆类型 ： |
Rabbit Polyclonal Antibody |
|
亚 型 ： |
Rabbit Ig |
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免 疫 原 ： |
KLH-conjugated synthetic peptide encompassing a sequence within the N-term region of human GTP-CH-1. |
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产品描述 ： |
GTP cyclohydrolase 1 (GTP-CH-1 or GCH-1) catalyzes the conversion of GTP to D-erythro-7,8-dihydroneopterin triphosphate, the first and rate-limiting step in tetrahydrobiopterin (BH4) biosynthesis. The final product (BH4) is an essential co-factor for three aromatic amino acid monooxygenases (F, Y, and W). Isoform GCH-1 is the functional enzyme. GTP-CH-1 positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVEC). GTP-CH-1 deficiency is a cause of malignant hyperphenylalaninemia due to tetrahydrobiopterin deficiency. It is also responsible for defective neurotransmission due to depletion of the neurotransmitters dopamine and serotonin. GTP-CH-1 (DYT5) is a cause for dystonia 5, a DOPA-responsive dystonia, defined by sustained involuntary muscle contractions. |
| 适用物种 ： |
H, M, R |
|
应用范围 ： |
E, WB, IHC |
| 应用说明 ： |
E: 1:500-1:1,000; WB: 1:200-1:500; IHC: 1:200-1:500 |
| 别 名 ： |
GCH-1; GTP cyclohydrolase I; GTP-CH-1; GTP cyclohydrolase 1; GTP-CH-I; GCH1; DYT5; GCH |
|
形 态 ： |
Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4, 0.1 mg/ml BSA, 25% glycerol with 0.09% sodium azide. Antibody was purified by Protein-A affinity chromatography. |
| 保存条件 ： |
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment. |
|
产品文献 ： |
[1] Blecharz KG et al. 2014. Biol Cell. 106(7): 219-35. PMID# 24749543. [2] Meng C et al. 2013. Neurological Sciences. 10.1007/s10072-013-1353-5. PMID# 23515624. |
| 产品链接 ： |
http://www.abbiotec.com/antibodies/gtp-ch-1-antibody |
| 标 记 物 ： |
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产品说明 ： |
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