FBN1 and Weill-Marchesani syndrome: Although the target substrate of ADAMTS17 is not yet clearly defined, as mutations in ADAMTS10 (OMIM: 608990), ADAMTS17, and FBN1 (OMIM: 134797) can all lead to Weill-Marchesani syndrome (WMS) and ADAMTS10 promotes microfibril biogenesis through direct interaction with fibrillin-1, we hypothesized that ADAMTS17 promotes microfibril biogenesis through interaction with fibrillin fibers21–27.