Anti-COL6A3 antibody
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概述
- 产品描述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
- 产品名称Anti-COL6A3 antibody
- 种属反应性Human,Mouse
- 验证应用IHC-P,IF
- 抗体类型兔多抗
- 免疫原The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310
- 偶联Non-conjugated
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性能
- 形态Liquid
- 浓度1 mg/mL.
- 存放说明Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
- 存储缓冲液Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 亚型IgG
- 纯化方式Immunogen affinity purified.
- 其它名称COL6A3 antibody; Collagen alpha-3(VI) chain antibody;
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应用
IHC-P: 1:100-1:300
IF: 1:100-1:500
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