COL6A3 rabbit pAb - ES4748

COL6A3 rabbit pAb

Sizes: 50μL, 100μL

Catalogue Numbers: ES4748-50, ES4748-100

Citations, Manuals and MSDS Available upon request.

Background: 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

Alternate Name: COL6A3; Collagen alpha-3 (VI) chain

Source: Rabbit

Applications: IHC; IF; ELISA

Dilution: Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications.

Reactivity: Human; Mouse

Immunogen: The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310

Storage and Stability: -20°C/1 year

Clonality: Polyclonal

Isotype: IgG

Concentration: 1 mg/ml

Human Gene ID: 1293

Human SWISS Prot NO: P12111

Subcellular Location: Secreted, extracellular space, extracellular matrix.

Research Use Only