Chronic Treatment with a Broad-Spectrum Metalloproteinase Inhibitor, Doxycycline, Prevents the Development of Spontaneous Aortic Lesions in a Mouse Model of Vascular Ehlers-Danlos Syndrome

There is no proven therapy or prevention for vascular Ehlers-Danlos syndrome (vEDS), a genetic disorder associated with the mutation of procollagen type III and characterized by increased fragility of vascular and hollow organ walls. Heterozygous COL3A1-deficient (HT) mice recapitulate a mild presentation of one of the variants of vEDS: haploinsufficiency for collagen III. Adult HT mice are characterized by increased metalloproteinase...