Invitrogen™ COL6A3 Polyclonal Antibody

Catalog No.	Quantity
PIPA5115038	100 μL

Catalog No. PIPA5115038 Supplier Invitrogen™ Supplier No. PA5115038

Description

Rabbit Polyclonal Antibody

Antibody detects endogenous levels of total Collagen VI alpha 3.

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].

Specifications

• Antigen: COL6A3
• Applications: Western Blot, Immunocytochemistry
• Classification: Polyclonal
• Concentration: 1 mg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 50% glycerol and 0.02% sodium azide
• Gene: COL6A3
• Gene Accession No.: P12111
• Gene Alias: AI507288; BTHLM1; CO6A3; COL6A3; Col6a-3; collagen alpha 3 chain type VI; collagen alpha3(VI); collagen alpha-3(VI) chain; collagen type VI alpha 3 chain; collagen VI, alpha-3 polypeptide; collagen, type VI, alpha 3; DYT27; procollagen, type VI, alpha 3; type VI collagen alpha 3 subunit; UCMD1
• Gene Symbols: COL6A3
• Host Species: Rabbit
• Immunogen: A synthesized peptide derived from human COL6A3(Accession P12111), corresponding to amino acid residues D2576-S2626.
• Purification Method: Affinity chromatography
• Quantity: 100 μL
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 1293
• Target Species: Human
• Content And Storage: -20°C
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG

Safety and Handling

WARNING: Cancer - www.P65Warnings.ca.gov