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COL6A3 rabbit pAb
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ES4748
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50 µL $148.00
100 µL $248.00
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Overview

Product name: COL6A3 rabbit pAb
Reactivity: Human;Mouse
Alternative Names: COL6A3; Collagen alpha-3(VI) chain
Source: Rabbit
Dilutions: Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications.
Immunogen: The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310
Storage: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
Concentration: 1 mg/ml
Molecular Weight: 344kD
GeneID: 1293
Human Swiss-Prot No: P12111
Cellular localization: Secreted, extracellular space, extracellular matrix .
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