产品简要
公司名称 :
赛默飞世尔
其他品牌 :
NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience
产品类型 :
抗体
产品名称 :
COL6A3重组兔单克隆抗体(118)
目录 :
MA5-30262
规格 :
100微升
价格 :
美国389.00
克隆性 :
重组
宿主 :
domestic rabbit
共轭标签 :
未共轭
克隆名称 :
118
反应物种 :
人类
应用 :
酶联免疫吸附测定
更多信息或购买 :
产品信息
产品类型 :
抗体
产品名称 :
COL6A3重组兔单克隆抗体(118)
目录# :
MA5-30262
规格 :
100微升
价格 :
美国389.00
克隆性 :
重组单克隆
纯度 :
蛋白质A
宿主 :
兔
反应物种 :
人类
应用 :
ELISA: 1:5, 000-1:10, 000
物种 :
人类
克隆 :
118
抗体亚型 :
IgG
储存 :
短期存放在4°C。 为长期储存, 存放在-20°C, 避免反复冷冻/解冻
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 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. [provided by RefSeq, Jun 2009].
免疫原 :
重组人类COL6A3/胶原VI蛋白
格式 :
液体
应用w/稀释 :
ELISA: 1:5, 000-1:10, 000
别名 :
AI507288; BTHLM1; CO6A3; COL6A3; Col6a-3; collagen alpha 3 chain type VI; collagen alpha3(VI); collagen alpha-3(VI) chain; collagen type VI alpha 3 chain; collagen VI, alpha-3 polypeptide; collagen, type VI, alpha 3; DYT27; procollagen, type VI, alpha 3; type VI collagen alpha 3 subunit; UCMD1
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公司信息
赛默飞世尔