GTP Cyclohydrolase 1 (GCH1) (N-Term) 抗体

ABIN1584545 产品详细信息, 供应商: Log in to see
抗原
  • DYT14
  • DYT5
  • DYT5a
  • GCH
  • GTP-CH-1
  • GTPCH1
  • HPABH4B
  • GTP-CH
  • GTPCH
  • Gch
  • MGC89622
  • gch1a
  • wu:fc16b10
  • zgc:195269
  • GTP cyclohydrolase 1
  • GTP cyclohydrolase 1-like
  • GCH1
  • Gch1
  • gch1
  • Weevi_1190
  • Halhy_0838
  • Sinme_1220
  • Sphch_1077
  • LOC100136794
抗原表位
N-Term
14
11
10
9
7
7
5
3
2
2
2
2
1
1
1
适用
人, 小鼠, 大鼠
82
31
30
5
5
4
3
3
2
2
2
2
2
1
1
1
宿主

55
17
12
克隆类型
多克隆
标记
非结合性
7
6
4
3
3
3
1
1
1
1
1
1
1
1
1
1
1
应用范围
ELISA, Immunohistochemistry (IHC), Western Blotting (WB)
68
49
27
13
8
8
7
1
1
选项
Supplier
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Supplier Product No.
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免疫原 KLH-conjugated synthetic peptide encompassing a sequence within the N-term region of human GTP-CH-1.
Blocking Peptide Blocking peptide for this product available: ABIN1607065
纯化方法 Antibody was purified by Protein-A affinity chromatography.
别名 GTP-CH-1 (GCH1 Antibody 摘要)
背景

Alternate Names: GCH-1, GTP cyclohydrolase I, GTP-CH-1, GTP cyclohydrolase 1, GTP-CH-I, GCH1, DYT5, GCH

Description: 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.

UniProt P30793
应用备注 E: 1:500-1:1,000, WB: 1:200-1:500, IHC: 1:200-1:500
限制 仅限研究用
状态 Liquid
浓度 1 mg/mL
缓冲液 Each vial contains 0.1 mg IgG in 0.1 mL (1 mg/mL) of PBS pH 7.4, 0.1 mg/mL BSA, 25 % glycerol with 0.09 % sodium azide.
储存液 Sodium azide
注意事项 Warning: This product contains sodium azide, which has been classified as Xn (Harmful), in European Directive 67/548/EEC in the concentration range of 0.1 - 1.0 %. When disposing of this reagent through lead or copper plumbing, flush with copious volumes of water to prevent azide build-up in drains.
注意事项 Minimize freeze-thaw cycles.
储存条件 -20 °C
储存方法 Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
有效期 12 months
厂商提供的图像
Immunohistochemistry (IHC) image for anti-GTP Cyclohydrolase 1 (GCH1) (N-Term) antibody (ABIN1584545) anti-GTP Cyclohydrolase 1 (GCH1) (N-Term) antibody
有引用在: Meng, Liang, Li, Chen, Liu, Li: "Changes of GTP cyclohydrolase I and neuronal apoptosis in rat spinal dorsal cord induced by sciatic nerve injury." in: Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, Vol. 34, Issue 12, pp. 2145-50, 2014 (PubMed).

Blecharz, Burek, Bauersachs, Thum, Tsikas, Widder, Roewer, Förster: "Inhibition of proteasome-mediated glucocorticoid receptor degradation restores nitric oxide bioavailability in myocardial endothelial cells in vitro." in: Biology of the cell, Vol. 106, Issue 7, pp. 219-35, 2014 (PubMed).

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