WB
Western blot analysis of COL6A3 expression in extracts of human skeletal muscle (A), human thymus (B), and human heart tissues(C).WB
Western blot analysis of COL6A3 expression in human thymus (A) and Hs67 (B) whole cell lysate.| Product Name | Mouse Monoclonal antibody to COL6A3 |
|---|---|
| Antibody Type | Primary Antibodies |
| Immunogen | raised against amino acids 301-600 mapping within an internal region of Collagen a3 Type VI of human origin. |
| Clonality | monoclonal |
|---|---|
| Isotype | IgG1, k |
| Host Species | Mouse |
| Tested Applications | WB |
| WB:1:100-1:1000 |
|
| Species Reactivity | Human |
| Concentration | 0.2mg/ml |
| Purification | Affinity purified |
| Gene Symbol | COL6A3 |
|---|---|
| Gene Synonyms | DYT27 UCMD1 BTHLM1 UCMD1C BTHLM1C |
| Gene Full Name | collagen type VI alpha 3 chain |
| Gene Summary | 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 autosomal recessive congenital myopathy that is more severe than Bethlem myopathy. Multiple transcript variants have been identified, but the full-length nature of only some of these variants has been described. [provided by RefSeq, Jun 2009] |
| Molecular Weight(MW) | 260-300 kDa |
| Cellular Localization | Extracellular matrix,Secreted. |
WB
Western blot analysis of COL6A3 expression in extracts of human skeletal muscle (A), human thymus (B), and human heart tissues(C).
WB
Western blot analysis of COL6A3 expression in human thymus (A) and Hs67 (B) whole cell lysate.| Application Notes | WB:1:100-1:1000 |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Store at 4° C, **DO NOT FREEZE**. Stable for one year from the date of shipment. Non-hazardous. No MSDS required. |
| Storage Buffer | Each vial contains 200 µg IgG1 kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin. |
