COL6A3 Polyclonal Antibody

SKU: BT-AP02094

Size:100μL

Background:COL6A3 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.

Research_area:Signal Transduction

Target_protein:COL6A3

applications:IHC-p, IF, ELISA

Reactivity:Human, Mouse

Clonality:Polyclonal

Clone ID:

Host:Rabbit

Isotype:IgG

Gene Symbol/ Name:COL6A3 Antibody

Immunogen:The antiserum was produced against synthesized peptide derived from human AMPK alpha around the phosphorylation site of Thr172. AA range:140-189

Storage:-20°C for 1 year

Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Concentration:1 mg/ml

Molecular weight(Da):343552

UniProt accession:Human: P12111

Synonyms:COL6A3; Collagen alpha-3(VI) chain

GeneID:Human: 1293