COL6A3 rabbit pAb

Name: COL6A3 rabbit pAb
Brand: ELK Biotechnology
Price: 148.0 USD
Availability: InStock

Vendor: ELK Biotechnology

SKU: ES4748

$148.00 USD

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For research use only — not intended for clinical or diagnostic purposes.

Product Overview

Applications

IHC;IF;ELISA

Cellular localization

Secreted, extracellular space, extracellular matrix .

Clonality

Polyclonal

Concentration

1 mg/ml

Geneid human

1293

Human swiss prot no

P12111

Immunogen

The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310

Isotype

IgG

Product weight

0.005kg

Reactivity

Human;Mouse

Recommended dilutions

Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications.

Source

Rabbit

Storage

-20°C/1 year

Background

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 a

COL6A3 rabbit pAb

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