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Antibodies

COL6A3 rabbit pAb

Product Sizes
50UL
£ POA
ES4748-50UL
100UL
£ POA
ES4748-100UL
About this Product
SKU:
ES4748
Additional Names:
COL6A3; Collagen alpha-3(VI) chain
Application:
Immunohistochemistry, Immunofluorescence, ELISA
Clonality:
Polyclonal
Concentration:
1 mg/ml
Extra Details:
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
Immunogen:
The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310
Isotype:
IgG
Reactivities:
Human, Mouse
Shipping Conditions:
Blue Ice
Source:
Rabbit
Storage Conditions:
-20[o]C
Supplier:
ELK Biotechnology
Type:
Antibodies: Polyclonal Antibody
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