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COL6A3 rabbit pAb
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ES4748
规格： 价格：
50µL ￥1280.00
100µL ￥1980.00
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Overview

产品名称: COL6A3 rabbit pAb
反应性: Human;Mouse
别名: COL6A3; Collagen alpha-3(VI) chain
宿主: Rabbit
稀释比: Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications.
免疫原: The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310
储存条件: -20°C/1 year
克隆性: Polyclonal
抗体亚型: IgG
浓度: 1 mg/ml
分子量: 344kD
基因ID: 1293
蛋白序列: P12111
细胞定位: Secreted, extracellular space, extracellular matrix .
研究背景: 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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