{"product_id":"es4748","title":"COL6A3 rabbit pAb","description":"\u003cstrong\u003eApplications:\u003c\/strong\u003e IHC;IF;ELISA\u003cbr\u003e\u003cstrong\u003eReactivity:\u003c\/strong\u003e Human;Mouse\u003cbr\u003e\u003cstrong\u003eSource:\u003c\/strong\u003e Rabbit\u003cbr\u003e\u003cstrong\u003eDilution:\u003c\/strong\u003e Immunohistochemistry: 1\/100 - 1\/300. Immunofluorescence: 1\/200 - 1\/1000. ELISA: 1\/40000. Not yet tested in other applications.\u003cbr\u003e\u003cstrong\u003eImmunogen:\u003c\/strong\u003e The antiserum was produced against synthesized peptide derived from human Collagen VI alpha3. AA range:2261-2310\u003cbr\u003e\u003cstrong\u003eStorage_stability:\u003c\/strong\u003e -20°C\/1 year\u003cbr\u003e\u003cstrong\u003eClonality:\u003c\/strong\u003e Polyclonal\u003cbr\u003e\u003cstrong\u003eIsotype:\u003c\/strong\u003e IgG\u003cbr\u003e\u003cstrong\u003eConcentration:\u003c\/strong\u003e 1 mg\/ml\u003cbr\u003e\u003cstrong\u003eHuman_gene_id:\u003c\/strong\u003e 1293\u003cbr\u003e\u003cstrong\u003eHuman_swiss_prot_no:\u003c\/strong\u003e P12111\u003cbr\u003e\u003cstrong\u003eSubcellular_location:\u003c\/strong\u003e Secreted, extracellular space, extracellular matrix .\u003cbr\u003e\u003cstrong\u003eOther_name:\u003c\/strong\u003e COL6A3; Collagen alpha-3(VI) chain\u003cbr\u003e\u003cstrong\u003eBackground:\u003c\/strong\u003e 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","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":50411365597464,"sku":"ES4748","price":250.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/ES4748-c-1.jpg?v=1751082407","url":"https:\/\/danabiosci.com\/products\/es4748","provider":"Dana Bioscience","version":"1.0","type":"link"}