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1型神经纤维瘤抗体 田源一抗
点击次数:140发布时间:2012/8/9 11:51:42
更新日期:2013/6/7 10:32:23
所 在 地:其它
产品型号:Anti-NF1/Neurofibromin 1
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详细内容
1型神经纤维瘤抗体Anti-NF1/Neurofibromin 1Neurofibromatosis Noonan syndrome; Neurofibromatosis related protein NF 1; Neurofibromatos
360TIYO.COM-上海田源公司
性能参数:
中文名称:1型神经纤维瘤抗体
英文名称:Anti-NF1/Neurofibromin 1
别名:Neurofibromatosis Noonan syndrome; Neurofibromatosis related protein NF 1; Neurofibromatos
包装号:G320
产地:中国大陆/进口产品
商标/品牌:Santa、Abcam、MBL、BD、Cell Signaling Technology
供应商:田源试剂 TR、新试剂 NR
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产品详细描述
产品介绍:This gene product appears to function as a negative regulator of the ras signal transduction pathway. Mutations in this gene have been linked to neurofibromatosis type 1, juvenile myelomonocytic leukemia and Watson syndrome. The mRNA for this gene is subject to RNA editing (CGA>UGA->Arg1306Term) resulting in premature translation termination. Alternatively spliced transcript variants encoding different isoforms have also been described for this gene. [provided by RefSeq, Jul 2008].神经纤维素蛋白首先发现于神经细胞,是一种肿瘤抑制蛋白,通过调控Ras基因控制异常细胞生长,并且在cAMP信号传导通路中起调节作用.神经纤维瘤Ⅰ型(neurofibromatosis type 1,NF1)是一种由内分泌紊乱引起的神经纤维瘤,属于常染色体显性遗传病,其发病率为1/3500,主要表现为咖啡斑、神经纤维瘤、Lisch结节(虹膜错构瘤)等。每3,500个新生儿中就有一个是神经纤维细胞瘤I型患者,其临床表现为表皮或皮下多发性神经纤维瘤,良性多于恶性,常沿神经干分布。有时,神经纤维瘤会长大,或者发展到脑和脊髓,大约有一半以上患者智力低下。1型神经纤维瘤抗体的质量标准:
■其它名称: | Neurofibromatosis Noonan syndrome; Neurofibromatosis related protein NF 1; Neurofibromatos |
■交叉反应: | hu, mo, rat, Rb, hrs, dog |
■产品类型: | 一抗 |
■应用领域: | /肿瘤/细胞生物/免疫学/神经生物学/信号转导/转录调节因子/G蛋白偶联受体/细胞骨架 |
■蛋白分子: | predicted molecular weight: 319kDa |
■浓度值: | 1mg/1ml |
■物理性状: | Lyophilized or Liquid |
■免疫原: | KLH conjugated synthetic peptide derived from human Neurofibromin 1 C-terminus |
■亚型: | polyclonal |
■纯化方法: | affinity purified by Protein A |
■保存液: | 0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide |
■应用技术: | //WB=1:100-500//ELISA=1:500-1000//IP=1:20-100//IHC-P=1:100-500//IHC-F=1:100-500//IF=1:100-500(石蜡切片需做抗原修复)/not yet tested in other applications./optimal dilutions/concentrations should be determined by the end user. |
■保存环境: | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
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1型神经纤维瘤抗体Anti-NF1/Neurofibromin 1的相关产品目录:
1mg/1ml 1型神经纤维瘤抗体 Anti-NF1/Neurofibromin 1 Neurofibromatosis Noonan syndrome; Neurofibromatosis related protein NF 1; Neurofibromatos的来源为Rabbit,其相关产品:免疫组化常用试剂免疫印迹常用试剂各种标记二抗
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