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Cell Signal Transduction Research Antibodies
COL6A3 Polyclonal AntibodyAntibody
Polyclonal, Rabbit
$225/ 100ul
QuantityTotal: $225
Ship worldwide. Contact order@enogene.com for ordering and bulk pricing or contact your local distributor.
Product Information
Molecular Weight343552Da
Protein NameCollagen alpha-3(VI) chain
Human Gene Id1293
Human Swiss Prot NoP12111
SpecificityCOL6A3 Polyclonal Antibody detects endogenous levels of COL6A3 protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Concentration1 mg/ml
Storage Stability-20°C/1 year
Other NameCOL6A3; Collagen alpha-3(VI) chain
Functiondisease: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 collage
Subcellular Locationextracellular region,proteinaceous extracellular matrix,collagen trimer,collagen type VI trimer,extracellular space,endoplasmic reticulum lumen,extracellular matrix,sarcolemma,extracellular exosome,extracellular vesicle,
ExpressionColon endothel,Fibroblast,Human uterus,Kidney,Liver,Placenta,Plasma,Pooled,
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
ImmunogenThe antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310
Gene NameCOL6A3
DilutionImmunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications.
Backgroundcollagen 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
Conjugated Antibody
This product is also available conjugated. Format: E20-71036-Conjugate. Price: $485 / Size: 100μl.
E20-71036-FITC$485 / 100μlE20-71036-Biotin$485 / 100μlE20-71036-HRP$485 / 100μlE20-71036-AP$485 / 100μlE20-71036-AF350$485 / 100μlE20-71036-AF405M$485 / 100μlE20-71036-AF488$485 / 100μlE20-71036-AF514$485 / 100μlE20-71036-AF532$485 / 100μlE20-71036-AF555$485 / 100μlE20-71036-AF568$485 / 100μlE20-71036-AF405S$485 / 100μlE20-71036-AF405L$485 / 100μlE20-71036-AF546$485 / 100μlE20-71036-AF594$485 / 100μlE20-71036-AF610$485 / 100μlE20-71036-AF635$485 / 100μlE20-71036-AF647$485 / 100μlE20-71036-AF680$485 / 100μlE20-71036-AF700$485 / 100μlE20-71036-AF750$485 / 100μlE20-71036-AF790$485 / 100μlE20-71036-APC$485 / 100μlE20-71036-PE$485 / 100μlE20-71036-Cy3$485 / 100μlE20-71036-Cy5$485 / 100μlE20-71036-Cy5.5$485 / 100μlE20-71036-Cy7$485 / 100μlE20-71036-PE-Cy5$485 / 100μl
Contact order@enogene.com to order conjugated formats. Magnetic Beads (1-30μm) conjugation is also available.
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