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Summary Type III collagen, [a1(III)]3 ,is an approx. 300 kDa molecule, found predominantly in skin, blood vessels, liver, placenta, tongue, and thymus. Collagen type III forms cofibrils with type I and/or V collagens in a number of tissues of mesenchymal origin, such as skin, tendon, ligaments, and bone. This collagen type is involved, directly or indirectly in several genetic diseases, including Ehlers-Danlos type IV disease.