Invitrogen™ Human COL6A3 (aa 2733-2878) Control Fragment Recombinant Protein

Product Code. 30199796

Brand: Invitrogen™ RP102437

Description

Recombinant Protein

Highest antigen sequence indentity to the following orthologs: Mouse (85%), Rat (85%). This recombinant protein control fragment may be used for blocking experiments. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.

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].

Specifications

• Accession Number: P12111
• Concentration: ≥5.0 mg/mL
• For Use With (Application): Blocking Assay, Control
• Formulation: 1 M urea, PBS with no preservative; pH 7.4
• Gene ID (Entrez): 1293
• Name: Human COL6A3 (aa 2733-2878) Control Fragment
• Quantity: 100 μL
• Regulatory Status: RUO
• Gene Alias: AI507288; BTHLM1; CO6A3; COL6A3; Col6a-3; collagen alpha 3 chain type VI; collagen alpha3(VI); collagen alpha-3(VI) chain; collagen type VI alpha 3 chain; collagen VI, alpha-3 polypeptide; collagen, type VI, alpha 3; DYT27; procollagen, type VI, alpha 3; type VI collagen alpha 3 subunit; UCMD1
• Common Name: COL6A3
• Gene Symbol: COL6A3
• Conjugate: Unconjugated
• Species: Human
• Recombinant: Recombinant
• Protein Tag: His-ABP-tag
• Sequence: PRDLKIVVLMLTGEVPEQQLEEAQRVILQAKCKGYFFVVLGIGRKVNIKEVYTFASEPNDVFFKLVDKSTELNEEPLMRFGRLLPSFVSSENAFYLSPDIRKQCDWFQGDQPTKNLVKFGHKQVNVPNNVTSSPTSNPVTTTKPVT
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Expression System: E. coli
• Form: Liquid
• Purity or Quality Grade: >80% by SDS-PAGE and Coomassie blue staining