FBN1 and Weill-Marchesani syndrome: Finally, we analyzed endogenous COL6 distribution and ECM deposition in dermal fibroblasts isolated from a patient with WMS due to a ADAMTS17 Thr343Ala mutation, where we previously showed intracellular retention and reduced ECM deposition of fibronectin, fibrillin-1, and COL1 (Karoulias et al, 2020a).