| Product Name | COL6A3 |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | COL6A3 antibody; Collagen alpha-3(VI) chain antibody; |
| Product description | 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 a |
| Immunogen | The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310 |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | IHCIF |
| IHC:1:100-1:300 IF:1:100-1:500 |
|
| Species Reactivity | HumanMouse |
| Concentration | 1mg/ml |
| Alternative Names | COL6A3 antibody Collagen alpha-3(VI) chain antibody |
|---|---|
| Molecular Weight(MW) | kDa |
| SwissProt ID | P12111 |
|---|
| Positive Control | Colon endothel,Fibroblast,Human uterus,Kidney,Liver,Placenta,Plasma,Pooled, |
|---|---|
| Application Notes | IHC:1:100-1:300 IF:1:100-1:500 |
| Form | Liquid |
|---|---|
| Storage Instructions | Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles. |
| Storage Buffer | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
