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COL6A3 Polyclonal Antibody

CAT: 0749-ABP53749-01Size: 30 µLDry Ice: NoHazardous: No
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CAT#:0749-ABP53749-01Size:30 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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.
Description
Rabbit Anti-COL6A3 Polyclonal Antibody
CAS Number
9007-83-4
Product Name Alternative
COL6A3; Collagen alpha-3 (VI) chain
Gene ID
1293
Accession Number
P12111
Reactivity
Human, Mouse
Immunogen
Synthesized peptide derived from the Internal region of human COL6A3 at AA rangle: 2230-2310
Clonality
Polyclonal
Tag
Collagen alpha-3 (VI) chain
Applications
IHC, IF, ELISA
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
1 mg/ml
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.
Shipping Conditions
Gel pack with blue ice.
Storage Conditions
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.
Notes
COL6A3 Polyclonal Antibody detects endogenous levels of COL6A3 protein.
Formulation
Liquid solution
Buffer pH
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Applications Notes
Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: IHC 1:100-1:300; IF 1:200-1:1000; ELISA 1:40000; Not yet tested in other applications.
Other Product Names
COL6A3; Collagen alpha-3 (VI) chain
Tested Applications
WB, IHC, IF, IP, ELISA
Host or Source
Rabbit
Accession Number URL
http://www.uniprot.org/uniprot/P12111
Isotype
Rabbit IgG

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