| 别名 | Collagen alpha-1(VI) chain |
| 基因名称 | Endogenous |
| 蛋白名称 | COL6A1 |
| 推荐应用 | |
| 反应物种 | COL6A1 |
| 存储缓冲液 | IHC 1:200-1:1000;WB 1:2000-1:10000;IF 1:200-1:1000;ELISA 1:5000-1:20000; |
| Human Gene Link | COL6A1;Collagen alpha-1;VI;chain |
| Human Swissprot No. | 1291 |
| Mouse Gene ID | P12109 |
| Mouse Swissprot No. | 12833 |
| 特异性 | Monoclonal |
| 稀释度 | Unmodified |
| 参考分子量 | 109kD |
| 预测分子量 | WB,IHC,IF,ELISA |
| 运输及保存条件 | Protein A |
| 宿主 | 147kD |
| 同种型 | Rabbit |
| 背景介绍 | The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy. [provided by RefSeq, Jul 2008], |
| 细胞定位 | Secreted, extracellular space, extracellular matrix . |
| 功能 | Disease:Defects in COL6A1 are a cause of Bethlem myopathy (BM) [MIM:158810]. BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles.,Function:Collagen VI acts as a cell-binding protein.,PTM:Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,similarity:Belongs to the type VI collagen family.,similarity:Contains 3 VWFA domains.,subunit:Trimers composed of three different chains: alpha-1(VI), alpha-2(VI), and alpha-3(VI) or alpha-5(VI) or alpha-6(VI)., |
| 纯化 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Clonality | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 修饰类型 | IgG,Kappa |
| 细胞定位 | Secreted, extracellular space, extracellular matrix . |
| 组织表达 | Blood,Brain,Fetal brain cortex,Fibroblast,Kidney,Liver,Ovary,Placenta, |
| 研究领域 | >>PI3K-Akt signaling pathway;>>Focal adhesion;>>ECM-receptor interaction;>>Protein digestion and absorption;>>Human papillomavirus infection |
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