COL6a3 Protein

Produktnummer ABIN7317153

Produktdetails

Target: COL6a3 (Collagen, Type VI, alpha 3 (COL6a3))

Protein-Typ: Recombinant

Spezies: Human

Quelle: HEK-293 Cells

Verwendungszweck: Recombinant Human COL6A3/Collagen-VI Protein

Sequenz: Thr3101-Thr3177

Produktmerkmale: A DNA sequence encoding the human COL6A3 (NP_004360.2) (Thr3101-Thr3177) was expressed.

Reinheit: > 95 % as determined by reducing SDS-PAGE.

Endotoxin-Niveau: < 1.0 EU per μg of the protein as determined by the LAL method.

Anwendungsinformationen

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Please refer to the printed manual for detailed information.

Buffer: Lyophilized from sterile PBS, pH 7.4

Lagerung: 4 °C,-20 °C,-80 °C

Informationen zur Lagerung: Generally, 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.

Antigendetails

Target: COL6a3 (Collagen, Type VI, alpha 3 (COL6a3))

Andere Bezeichnung: COL6A3/Collagen-VI (COL6a3 Produkte)

Synonyme: wu:fb06e12 Protein, wu:fs74g09 Protein, COL6A3 Protein, AI507288 Protein, Col6a-3 Protein, collagen type VI alpha 3 chain Protein, collagen, type VI, alpha 3 Protein, COL6A3 Protein, col6a3 Protein, Col6a3 Protein

Hintergrund:

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 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.

Synonym: COL6A3,Collagen-VI

Molekulargewicht: 8.5 kDa

NCBI Accession: NP_004360