Thermo Fisher Scientific COL6A3 Recombinant Rabbit Monoclonal Antibody (118)

Applications

• ELISA (ELISA)
  Tested Dilution: 1:5,000–1:10,000

Product Specifications

Product Specific Information

This product is preservative-free. It is recommended to add sodium azide to avoid contamination (final concentration 0.05%–0.1%).

Recombinant rabbit monoclonal antibodies are produced using in vitro expression systems. The antibody DNA sequences from immunoreactive rabbits are cloned and screened to select optimal clones for production.
Advantages include:

• Enhanced specificity and sensitivity
• Lot-to-lot consistency
• Animal origin-free formulation
• Broader immunoreactivity due to larger rabbit immune repertoire

This antibody has specificity for Human COL6A3/Collagen-VI.

Target Information

The COL6A3 gene encodes the alpha-3 chain of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain is significantly larger than the alpha-1 and -2 chains due to additional subdomains similar to von Willebrand Factor type A domains, located in the amino-terminal globular domain.
These domains interact with extracellular matrix proteins, contributing to matrix organization.

Mutations in COL6A3 are associated with:

• Bethlem myopathy (autosomal dominant proximal myopathy)
• Ullrich congenital muscular dystrophy, a more severe autosomal recessive form

Multiple transcript variants have been identified, though only some have been fully characterized.
[Provided by RefSeq, Jun 2009]

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