The discoveries that a rare connective tissue disorder, Weill-Marchesani syndrome, was genetically linked with recessive mutations of ADAMTS10 (WMS1; OMIM #277600) (Dagoneau et al., 2004) as well as dominant mutations of fibrillin-1 (WMS2; OMIM #608328) (Faivre et al., 2003) suggested that ADAMTS10 and fibrillin-1 (FBN1) might interact and/or act cooperatively in a common pathway. Here, FBN1 is linked to Weill-Marchesani syndrome.