Thermo Fisher Scientific COL6A3 Polyclonal Antibody

Applications

Western Blot (WB)

• Tested Dilution: 1:500–1:2,000

Immunocytochemistry (ICC/IF)

• Tested Dilution: 1:100–1:500

Product Specifications

Product Specific Information

Antibody detects endogenous levels of total Collagen VI alpha 3.

Target Information

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 due to an increased number of subdomains similar to von Willebrand Factor type A domains, found in the amino terminal globular domain of all alpha chains. These domains bind extracellular matrix proteins, explaining the role 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 also cause Ullrich congenital muscular dystrophy (Ullrich scleroatonic muscular dystrophy), an autosomal recessive congenital myopathy more severe than Bethlem myopathy. Multiple transcript variants have been identified, though only some full-length variants are described.
[provided by RefSeq, Jun 2009]

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.