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COL6A3 Polyclonal Antibody

COL6A3 Polyclonal Antibody

Views(64) Publications(0) Catalog no(ABP53749)
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Specification

Product name COL6A3 Polyclonal Antibody
Immunogen Synthesized peptide derived from the Internal region of human COL6A3 at AA rangle: 2230-2310
Host Rabbit
Reactivity Human, Mouse
Applications ELISA, IF, IHC-P
Applications notes Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: IHC-P (1:100-1:300), IF (1:200-1:1000), ELISA (1:40000). Not yet tested in other applications.
Clonality Polyclonal
Preparation method The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen
Alternative COL6A3; Collagen alpha-3(VI) chain

Product Properties

Formulation Liquid solution
Concentration 1 mg/ml
Storage buffer PBS containing 50% Glycerol, 0.5% BSA and 0.02% Sodium Azide.
Storage instructions Stable for one year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Aliquot to avoid repeated freezing and thawing.
Shipping Gel pack with blue ice.
Precautions The product listed herein is for research use only and is not intended for use in human or clinical diagnosis. Suggested applications of our products are not recommendations to use our products in violation of any patent or as a license. We cannot be responsible for patent infringements or other violations that may occur with the use of this product.

Additional Information

Background COL6A3 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.
Gene ID 1293
Alternative COL6A3; Collagen alpha-3(VI) chain
Others COL6A3 Polyclonal Antibody detects endogenous levels of COL6A3 protein.
Accession P12111

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