COL6A3, Rabbit, Polyclonal Antibody, Abnova™

Rabbit polyclonal antibody raised against synthetic peptide of COL6A3.

Brand:  Abnova PAB17517.100ug

Product Code. 16062957

Description

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

Specifications

• Antigen: COL6A3
• Classification: Polyclonal
• Description: Rabbit polyclonal antibody raised against synthetic peptide of COL6A3.
• Formulation: In PBS, pH 7.4 (150mM NaCl, 0.02% sodium azide, 50% glycerol)
• Gene Accession No.: P12111
• Gene Symbols: COL6A3
• Immunogen: A synthetic peptide corresponding to internal of human COL6A3.
• Regulatory Status: RUO
• Gene ID (Entrez): 1293
• Target Species: Human, Mouse
• Form: Liquid

• Applications: ELISA, Immunofluorescence, Immunohistochemistry (PFA fixed)
• Conjugate: Unconjugated
• Dilution: Immunohistochemistry (1:50-1:100) Immunofluorescence (1:500-1:1000) ELISA (1:40000) The optimal working dilution should be determined by the end user.
• Gene: COL6A3
• Gene Alias: DKFZp686D23123/DKFZp686K04147/DKFZp686N0262/FLJ34702/FLJ98399
• Host Species: Rabbit
• Quantity: 100 μg
• Primary or Secondary: Primary
• Test Specificity: This antibody detects endogenous levels of total COL6A3 protein.
• Content And Storage: Store at -20°C.
  Aliquot to avoid repeated freezing and thawing.

For Research Use Only