Thermo Fisher Scientific COL6A3 Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:1,000

-

ELISA (ELISA)

1:25,000-1:50,000

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Recombinant Human COL6A3/Collagen-VI protein

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Purification

Antigen affinity chromatography, Protein A

Storage buffer

PBS

Contains

no preservative

Storage conditions

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2866899

Product Specific Information

This product can be used in direct ELISA.

Target Information

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].

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.