Article
Type III collagen affects dermal and vascular collagen fibrillogenesis and tissue integrity in a mutant Col3a1 transgenic mouse model.
Matrix biology : journal of the International Society for Matrix Biology - 1 Sept 2018
D'hondt Sanne, Guillemyn Brecht, Syx Delfien, Symoens Sofie, De Rycke Riet, Vanhoutte Leen, Toussaint Wendy, Lambrecht Bart N, De Paepe Anne, Keene Douglas R, Ishikawa Yoshihiro, Bächinger Hans Peter, Janssens Sophie, Bertrand Mathieu J M, Malfait Fransiska
Abstract excerpt
Type III collagen is a major fibrillar collagen consisting of three identical α1(III)-chains that is particularly present in tissues exhibiting elastic properties, such as the skin and the arterial wall. Heterozygous mutations in the COL3A1 gene result in vascular Ehlers-Danlos syndrome (vEDS), a severe, life-threatening disorder, characterized by thin, translucent skin and propensity to arterial, intestinal and...
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