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COL6A3 Rabbit anti-Human, Mouse, Polyclonal, Invitrogen™

Rabbit Polyclonal Antibody

Manufacturer:  Invitrogen PA549914

Catalog No. PA549914


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Description

Description

The antibody detects endogenous levels of total COL6A3 protein.

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 domit 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. [provided by RefSeq, Jun 2009].
Specifications

Specifications

COL6A3
Polyclonal
Unconjugated
COL6A3
Liquid
COL6A3
Synthesized peptide derived from internal of human Collagen VI alpha3.
Antigen affinity chromatography
RUO
Antibody
Polyclonal
Human, Mouse
Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin)
1 mg/mL
PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
P12111
Collagen alpha-3(VI) chain, CO6A3,
Rabbit
IgG
100 μL
-20°C
Primary
12835, 1293
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