Rabbit Collagen VI Polyclonal Antibody | anti-COL6A1 antibody
Collagen VI Rabbit pAb
IHC: 1:50-1:200
ICC/IF: 1:50-1:200
NCBI and Uniprot Product Information
NCBI Description
The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy. [provided by RefSeq, Jul 2008]
Uniprot Description
COL6A1: Collagen VI acts as a cell-binding protein. Defects in COL6A1 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 COL6A1 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.
Protein type: Extracellular matrix; Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 21q22.3
Cellular Component: extracellular matrix; proteinaceous extracellular matrix; protein complex; membrane; lysosomal membrane; endoplasmic reticulum lumen; extracellular region; sarcolemma; collagen type VI
Molecular Function: platelet-derived growth factor binding
Biological Process: osteoblast differentiation; axon guidance; extracellular matrix disassembly; collagen catabolic process; extracellular matrix organization and biogenesis; cell adhesion
Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy
Research Articles on COL6A1
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Product Notes
The COL6A1 col6a1 (Catalog #AAA8542929) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Collagen VI Rabbit pAb reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Collagen VI can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF). WB: 1:1000-1:2000 IHC: 1:50-1:200 ICC/IF: 1:50-1:200. Researchers should empirically determine the suitability of the COL6A1 col6a1 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, 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
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