ITT1036
ITT1036
ITT1036
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- Catalog: ITT1036
- Gene/Protein: COL6A3
- Product Description: Immunotag™ COL6A3 Polyclonal Antibody
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Description
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Specifications
Antibody Specification Datasheet 
IMPORTANT NOTE This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. Target Protein COL6A3 Clonality Polyclonal Storage/Stability -20°C/1 year Application IHC-p,IF,ELISA Recommended Dilution Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications. Concentration 1 mg/ml Reactive Species Human,Mouse Host Species Rabbit Immunogen The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310 Specificity COL6A3 Polyclonal Antibody detects endogenous levels of COL6A3 protein. Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Gene Name COL6A3 Accession No. P12111 Alternate Names COL6A3; Collagen alpha-3(VI) chain Description collagen type VI alpha 3 chain(COL6A3) Homo sapiens 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 Cell Pathway/ Category Focal adhesion,ECM-receptor interaction, Protein Expression Colon endothel,Fibroblast,Human uterus,Kidney,Liver,Placenta,Plasma,Pooled, Subcellular Localization extracellular region,proteinaceous extracellular matrix,collagen trimer,collagen type VI trimer,extracellular space,endoplasmic reticulum lumen,extracellular matrix,sarcolemma,extracellular exosome,extracellular vesicle, Protein Function disease:Defects in COL6A3 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 COL6A3 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.,PTM:The N-terminus is blocked.,similarity:Belongs to the type VI collagen family.,similarity:Contains 1 BPTI/Kunitz inhibitor domain.,similarity:Contains 1 fibronectin type-III domain.,similarity:Contains 12 VWFA domains.,similarity:Contains 16 LRR (leucine-rich) repeats.,similarity:Contains 5 collagen-like domains.,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)., Usage For Research Use Only! Not for diagnostic or therapeutic procedures. - Reviews
| Antibody Specification | |
| Datasheet | |
| IMPORTANT NOTE | This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. |
| Target Protein | COL6A3 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | IHC-p,IF,ELISA |
| Recommended Dilution | Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse |
| Host Species | Rabbit |
| Immunogen | The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310 |
| Specificity | COL6A3 Polyclonal Antibody detects endogenous levels of COL6A3 protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | COL6A3 |
| Accession No. | P12111 |
| Alternate Names | COL6A3; Collagen alpha-3(VI) chain |
| Description | collagen type VI alpha 3 chain(COL6A3) Homo sapiens 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 |
| Cell Pathway/ Category | Focal adhesion,ECM-receptor interaction, |
| Protein Expression | Colon endothel,Fibroblast,Human uterus,Kidney,Liver,Placenta,Plasma,Pooled, |
| Subcellular Localization | extracellular region,proteinaceous extracellular matrix,collagen trimer,collagen type VI trimer,extracellular space,endoplasmic reticulum lumen,extracellular matrix,sarcolemma,extracellular exosome,extracellular vesicle, |
| Protein Function | disease:Defects in COL6A3 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 COL6A3 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.,PTM:The N-terminus is blocked.,similarity:Belongs to the type VI collagen family.,similarity:Contains 1 BPTI/Kunitz inhibitor domain.,similarity:Contains 1 fibronectin type-III domain.,similarity:Contains 12 VWFA domains.,similarity:Contains 16 LRR (leucine-rich) repeats.,similarity:Contains 5 collagen-like domains.,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)., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |