Invitrogen™ COL6A3 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA5112163

Catalog No. PIPA5112163

Description

This product can be used in direct ELISA.

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
• Classification: Polyclonal
• Formulation: PBS with no preservative
• Gene Accession No.: P12111
• Gene Symbols: COL6A3
• Immunogen: Recombinant Human COL6A3/Collagen-VI protein.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG

• Applications: ELISA, Western Blot
• Conjugate: Unconjugated
• Gene: COL6A3
• 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
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography, Protein A
• Regulatory Status: RUO
• Gene ID (Entrez): 1293
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid