Reduced expression of ADAMTS-1 in mice and humans has previously been associated with MFS-related aortopathy,35 but our most recent data point towards a greater role for ADAMTS-5, at least in mice.25 The contribution of proteoglycans and their remodeling to the pathology of MFS in aortas will require further investigation, especially since mutations in ADAMTS family members were found to phenocopy genetic disorders caused by mutations affecting FBN1. 53,54. This evidence concerns the gene ADAMTS5 and hereditary disease.