Rabbit anti-Human, Mouse Collagen VI alpha3 Polyclonal Antibody | anti-CO6A3 antibody
Collagen VI alpha3 Conjugated Antibody
AF350 conjugated: most applications: 1:50-1:250
AF405 conjugated: most applications: 1:50-1:250
AF488 conjugated: most applications: 1:50-1:250
AF555 conjugated: most applications: 1:50-1:250
AF594 conjugated: most applications: 1:50-1:250
AF647 conjugated: most applications: 1:50-1:250
AF680 conjugated: most applications: 1:50-1:250
AF750 conjugated: most applications: 1:50-1:250
Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
COL6A3: Collagen VI acts as a cell-binding protein. Defects in COL6A3 are a cause of Bethlem myopathy (BM). 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. Defects in COL6A3 are a cause of Ullrich congenital muscular dystrophy (UCMD); 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. Belongs to the type VI collagen family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted; Secreted, signal peptide; Extracellular matrix
Chromosomal Location of Human Ortholog: 2q37
Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; endoplasmic reticulum lumen; extracellular region; collagen type VI; vesicle; sarcolemma
Molecular Function: serine-type endopeptidase inhibitor activity
Biological Process: axon guidance; extracellular matrix disassembly; collagen catabolic process; muscle development; extracellular matrix organization and biogenesis; cell adhesion
Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy; Dystonia 27
Research Articles on CO6A3
Similar Products
Product Notes
The CO6A3 col6a3 (Catalog #AAA9445034) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Collagen VI alpha3 Conjugated Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's Collagen VI alpha3 can be used in a range of immunoassay formats including, but not limited to, Most Applications. Suggested Dilution: AF350 conjugated: most applications: 1:50-1:250 AF405 conjugated: most applications: 1:50-1:250 AF488 conjugated: most applications: 1:50-1:250 AF555 conjugated: most applications: 1:50-1:250 AF594 conjugated: most applications: 1:50-1:250 AF647 conjugated: most applications: 1:50-1:250 AF680 conjugated: most applications: 1:50-1:250 AF750 conjugated: most applications: 1:50-1:250 Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000. Researchers should empirically determine the suitability of the CO6A3 col6a3 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Collagen VI alpha3, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
