Although ADAMTS2 is a well-known procollagen-cleaving enzyme and a causative gene for Ehlers-Danlos syndrome (EDS), a skin disorder caused by abnormal collagen fibrillogenesis (Colige et al, 1999; Jaffey et al, 2019), Bekhouche et al recently revealed that TGF-β signaling is one of the targets of ADAMTS2 (Bekhouche et al, 2016). The gene discussed is TGFB1; the disease is Ehlers-Danlos syndrome.