COL6A2 Anticuerpo(K-15): sc-83608

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Collagens (COLs) are fibrous, extracellular matrix proteins with high tensile strength that function as the major components of connective tissue, such as tendons and cartilage. All COL proteins contain a triple helix domain and frequently show lateral self-association in order to form complex connective tissues. There are several types of COL proteins, including fibril-forming interstitial COLs (types I, II, III and V), basement membrane COLs (type IV) and beaded filament COLs (type VI). COL6A2 (collagen, type VI, alpha 2), also known as PP3610, is a 1,019 amino acid secreted protein that contains three VWFA domains and functions as the second of three alpha chains that comprise the type VI COL protein complex. Existing as a trimer with two other type VI alpha proteins, COL6A2 acts as a cell-binding protein that plays an important role in the organization of matrix components. Defects in the gene encoding COL6A2 are associated with Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). Multiple isoforms of COL6A2 exist due to alternative splicing events.