U-Blot® COL6A2 Rabbit mAb
Ucallm_WR5968-50
Price range: $268.00 through $328.00
Product details
Background：
This gene encodes one of the three alpha chains of type VI collagen , a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. 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 this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene. [provided by RefSeq , Jul 2008] ,
Specifications
| Size | 50μL/100μL |
| Target | COL6A2 |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | 109kD |
| MW(Observed) | 130kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1:1000; WB 1:500-1:2000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM1868R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | COL6A2 |
| Protein Name | Collagen alpha-2(VI) chain |
| Other Name | COL6A2; / Collagen alpha-2; / VI; / chain |
| Species | Human |
| Gene ID | 1292 |
| Uniprot | P12110, |
| Species.1 | Mouse |
| Gene ID.1 | 12834 |
| Uniprot.1 | Q02788 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Secreted, extracellular space, extracellular matrix . Membrane; Peripheral membrane protein . Recruited on membranes by CSPG4. |
| Tissue Expression | Fibroblast,Kidney,Liver,Ovary,Placenta,Uterus, |
| Signaling_pathway | Cellular Processes >> Cellular community - eukaryotes >> Focal adhesion;Environmental Information Processing >> Signal transduction >> PI3K-Akt signaling pathway;Environmental Information Processing >> Signaling molecules and interaction >> ECM-receptor interaction |
| Research Areas | >>PI3K-Akt signaling pathway; / >>Focal adhesion; / >>ECM-receptor interaction; / >>Protein digestion and absorption; / >>Human papillomavirus infection |
| Function | Disease:Defects in COL6A2 are a cause of Bethlem myopathy (BM) [MIM:158810]. BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles.,Disease:Defects in COL6A2 are a cause of Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]; also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy.,Function:Collagen VI acts as a cell-binding protein.,PTM:Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,similarity:Belongs to the type VI collagen family.,similarity:Contains 3 VWFA domains.,subcellular location:Recruited on membranes by CSPG4.,subunit:Trimers composed of three different chains: alpha-1(VI), alpha-2(VI), and alpha-3(VI) or alpha-5(VI) or alpha-6(VI). Interacts with CSPG4., |
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