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COL6A3 rabbit pAb

Ref. EK-ES4748

50µl
188.00 €
100µl
316.00 €
Estimated delivery in United States, on Wednesday 23 Oct 2024

Product Information

Name:
COL6A3 rabbit pAb
Synonyms:
  • COL6A3; Collagen alpha-3(VI) chain
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 a

Notice:
Our products are intended for lab use only. For any other use, please contact us.
Brand:
ELK Biotechnology
Long term storage:
Notes:

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Hazard Info

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