COL6A3 多克隆抗体-COL6A3 Polyclonal Antibody代理/参数/厂家-一抗-艾美捷科技有限公司

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背景资料: COL6A3 encodes the alpha-3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain of type VI collagen is much larger than the alpha-1 and -2 chains. This difference in size is largely due to an increase in the number of subdomains, similar to von Willebrand Factor type A domains, that are found in the amino terminal globular domain of all the alpha chains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in the type VI collagen genes are associated with Bethlem myopathy, a rare autosomal dominant proximal myopathy with early childhood onset. Mutations in this gene are also a cause of Ullrich congenital muscular dystrophy, also referred to as Ullrich scleroatonic muscular dystrophy, an autosomal recessive congenital myopathy that is more severe than Bethlem myopathy. Multiple transcript variants have been identified, but the full-length nature of only some of these variants has been described.
蛋白别名为：COL6A3; Collagen alpha-3(VI) chain;基因ID为：1293；蛋白质ID:P12111

应用类型: IHC-p，IF，ELISA
补充：最优的抗体稀释比例需要基于客户实验进行优化.建议的起始稀释比例如下: IHC-p: 1:100-1:300, IF: 1:200-1:1000, ELISA: 1:40000. Not yet tested in other applications.

保存建议: 厂家推荐蓝冰运输.在发货后能在-20℃保存1年.为最大限度的避免损失，请在打开管盖之前融化抗体并离心.我们建议使用前分装以避免反复冻融.
本产品为液体形式,溶解于PBS缓冲液中，含50%甘油，0.5%BSA和0.02%NaN3.

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