Figure 1
From: Fibrillin assemblies: extracellular determinants of tissue formation and fibrosis
Hypothetical models of MFS and SSS pathogenesis. The top drawing depicts the normal wild-type (WT) condition in which a cell interacts with fibrillin-1 microfibrils through RGD-mediated integrin binding and through TGF-β signals released from the fibrillin-1 microfibrils (thin arrows). Mutations in MFS decrease microfibril deposition/stability and promote excessive TGF-β signaling (thick arrow). Mutations in stiff skin syndrome perturb integrin-mediated microfibril deposition and increase matrix-directed TGF-β signaling (thick arrows).