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货号: PD6306
货期: 现货
价格: 50μL/960;100μL/1600;200μL/2560
所属分类:
靶点: COL6A3
应用: IHC,IF,ELISA
反应性 : Human,Mouse
宿主物种 : Rabbit
MW(CalcμLated) : 344kD
同种型-Isotype : IgG
隐藏域元素占位
详细信息
推荐稀释比 : IHC 1:100-1:300;IF 1:200-1:1000;ELISA 1:40000;Not yet tested in other applications.
组成 : Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺 : The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
储存 : -15°C to -25°C/1 year(Do not lower than -25°C
浓度 : 1 mg/ml
克隆性 : Polyclonal
抗原&靶点信息
特异性 : COL6A3 Polyclonal Antibody detects endogenous levels of COL6A3 protein.
基因名称 : COL6A3
蛋白名称 : Collagen alpha-3(VI) chain
别名 : COL6A3;Collagen alpha-3;VI;chain
背景:
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


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