The alpha-3 chain is one of the three alpha chains in type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain of type VI collagen is considerably larger than the alpha-1 and -2 chains. The increased number of subdomains, similar to von Willebrand Factor type A domains, that are found in the amino terminal globular domain of all alpha chains accounts for the variation in size. The expression of these genes has been linked to the binding of extracellular matrix proteins, which is an interaction that explains the significance of this collagen in matrix organization. Mutation of the type VI collagen genes is linked to Bethlem myopathy, a rare autosomal dominant proximal myopathy that begins in early childhood. This gene is also linked to Ullrich congenital muscular dystrophy, commonly known as Ullrich scleroatonic muscular dystrophy, an autosomal recessive congenital myopathy that is more severe than Bethlem myopathy. Several transcript variants have been discovered, but only some of them have been characterized in full. Human COL6A3/Collagen-VI Recombinant Protein is a highly pure recombinant protein developed by Assay Genie for use in a range of applications.
Product Name:
Human COL6A3/Collagen-VI Recombinant Protein (RPES3076)
Product Code:
RPES3076
Size:
20µg
Species:
Human
Expressed Host:
HEK293 Cells
Synonyms:
COL6A3,Collagen-VI
Accession:
NP_004360.2
Sequence:
Thr3101-Thr3177
Fusion tag:
Endotoxin:
<1.0 EU per µg of the protein as determined by the LAL method.
Protein Construction:
A DNA sequence encoding the human COL6A3 (NP_004360.2) (Thr3101-Thr3177) was expressed.
Purity:
> 95 % as determined by reducing SDS-PAGE.
Mol Mass:
8.5 kDa
Formulation:
Lyophilized from sterile PBS, pH 7.4
Shipping:
This product is provided as lyophilized powder which is shipped with ice packs.
Stability and Storage:
Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
