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Ⅵ型胶原α3(COL6a3)抗体
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。

产品名称
Anti-COL6A3
Anti-collagen type VI alpha 3 chain Antibody
产品介绍
靶标:

COL6A3


产品别名:

BTHLM1; DYT27; UCMD1; COL6A3; collagen type VI alpha 3 chain; collagen type VI alpha 3 chain; collagen alpha-3(VI) chain; collagen VI, alpha-3 polypeptide; collagen, type VI, alpha 3; epididymis secretory sperm binding protein; Ⅵ型胶原α3(COL6a3);


背景信息:
This gene 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 a

宿主:Rbt
类型:Pab
同种型:IgG  
应用:WB
纯化方式:亲和纯化
偶联物:Unconjugated
性状:液体
存储溶液:参阅说明书
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
稀释比例: Optimal dilutions/concentrations should be determined by the end user           
储存:经常使用则4°C保存。-20°C保存不超过两年。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。