产品说明
一般描述
Collagen alpha-3(VI) chain (UniProt P12111; also known as Collagen VI alpha-3 polypeptide) is encoded by the COL6A3 gene (Gene ID 1293) in human. Type VI collagen is an extracellular matrix (ECM) component present in virtually all connective tissues, including cartilage, bone, tendon, muscles and cornea, where it forms microfibrils in close association with basement membranes. In addition to anchoring the basement membrane to the pericellular matrix in muscle, research also indicates a role for collagen VI in cell signaling and cell migration. The basic structural unit of collagen VI is a heterotrimer composed of the alpha-1(VI), alpha-2(VI), and alpha-3(VI) chains (encoded by the COL6A1, COL6A2, and COL6A3 genes, respectively). The α1(VI) and α2(VI) chains are similar in size and domain structure, they contain a 335- or 336-amino acid triple helix region that is characteristic of all collagens. Flanking the triple helix are domains homologous to the A-type domains found in von Willebrand factor (VWA domains). α1(VI) and α2(VI) contain one VWA domain N-terminal to the triple helix (N1) and two VWA domains C-terminal of the helix (C1 and C2). The α3(VI) chain, on the other hand, is much larger with 10 N-terminal (N1–N10) and two C-terminal VWA domains (C1 and C2), and several other types of identifiable domains in the C terminal region (C3–C5). Mutations in the COL6A1, COL6A2, and COL6A3 genes are known causes of Ullrich congenital muscular dystrophy (UCMD) and Bethlem myopathy (BM). Three additional type VI collagen chains have been reported in 2008 (α4(VI), α5(VI) and α6(VI) chains encoded by COL6A4, COL6A5, and COL6A6, respectively).
免疫原
Purified human collagen VI.
应用
This Anti-Collagen VI alpha-3 Antibody, clone 3C4, Ascites Free is validated for use in Immunohistochemistry (Paraffin), Flow Cytometry, Immunocytochemistry, Immunoprecipitation, Electron Microscopy for the detection of Collagen VI alpha-3.
质量
Evaluated by Immunohistochemistry in human breast tissue.
Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected collagen VI alpha-3 chain immunoreactivity in human breast tissue section.
目标描述
343.7/321.4/113.2/278.2/134.7 kDa (isoform 1/2/3/4/5 pro-form) and 340.8/318.5/110.4/275.3/131.8 kDa (isoform 1/2/3/4/5 mature form) calculated
外形
Unpurified.
其他说明
Concentration: Please refer to lot specific datasheet.
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
eCl@ss | 32160702 |
NACRES | NA.41 |
产品性质
质量水平 | 100 |
生物来源 | mouse |
抗体形式 | culture supernatant |
antibody product type | primary antibodies |
克隆 | 3C4, monoclonal |
species reactivity | human |
technique(s) | electron microscopy: suitable flow cytometry: suitable immunocytochemistry: suitable immunohistochemistry: suitable (paraffin) immunoprecipitation (IP): suitable |
同位素/亚型 | IgG1κ |
NCBI登记号 | NP_004360 |
UniProt登记号 | P12111 |
运输 | dry ice |
Gene Information | human ... COL6A3(1293) |
安全信息
储存分类代码 | 10 - Combustible liquids |
WGK | WGK 1 |
Sigma-Aldrich