Target：COL6A1

Fields：PI3K-Akt signaling pathway;Focal adhesion;ECM-receptor interaction;Protein digestion and absorption;Human papillomavirus infection

Gene Name：COL6A1

Protein Name：Collagen alpha-1(VI) chain

Human Gene Id：1291

Human Swiss Prot No：P12109

Mouse Gene Id：12833

Mouse Swiss Prot No：Q04857

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.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：IHC 1:100 - 1:300. ELISA: 1:10000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：COL6A1;Collagen alpha-1(VI) chain

Molecular Weight(Da)：109kD

Background： 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],

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).,

Subcellular Location：Secreted, extracellular space, extracellular matrix .

Expression： Blood,Brain,Fetal brain cortex,Fibroblast,Kidney,Liver,Ovary,Placenta,