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ITT1034

ITT1034
  • Catalog: ITT1034
  • Gene/Protein: COL6A1
  • Product Description: Immunotag™ COL6A1 Polyclonal Antibody
385.0000
Price in reward points: 385

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Immunotag™ COL6A1 Polyclonal Antibody
Antibody Specification
Datasheet
Target Protein COL6A1
Clonality Polyclonal
Storage/Stability -20°C/1 year
Application IHC-p,ELISA
Recommended Dilution Immunohistochemistry: 1/100 - 1/300. ELISA: 1/10000. Not yet tested in other applications.
Concentration 1 mg/ml
Reactive Species Human,Mouse
Host Species Rabbit
Immunogen The antiserum was produced against synthesized peptide derived from human Collagen VI alpha1. AA range:191-240
Specificity COL6A1 Polyclonal Antibody detects endogenous levels of COL6A1 protein.
Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen
Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Gene Name COL6A1
Accession No. P12109 Q04857
Alternate Names COL6A1; Collagen alpha-1(VI) chain
Description collagen type VI alpha 1 chain(COL6A1) Homo sapiens 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],
Cell Pathway/ Category Focal adhesion,ECM-receptor interaction,
Protein Expression Blood,Brain,Fetal brain cortex,Fibroblast,Kidney,Liver,Ovary,Placenta,
Subcellular Localization extracellular region,collagen trimer,collagen type VI trimer,lysosomal membrane,endoplasmic reticulum lumen,membrane,extracellular matrix,sarcolemma,protein complex,extracellular exosome,
Protein Function 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).,
Usage For Research Use Only! Not for diagnostic or therapeutic procedures.
Material Safety Data Sheet
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