COL6A3 Recombinant Rabbit Monoclonal Antibody (118), Invitrogen™

Description

• 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 expression systems are developed by cloning in the specific antibody DNA sequences from immunoreactive rabbits
• Then, individual clones are screened to select the best candidates for production
• The advantages of using recombinant rabbit monoclonal antibodies include: better specificity and sensitivity, lot-to-lot consistency, animal origin-free formulations, and broader immunoreactivity to diverse targets due to larger rabbit immune repertoire
• This antibody has specificity for Human COL6A3/Collagen-VI
• 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 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].