Figure 7
From: Skeletal Dysplasia Mutations Effect on Human Filamins’ Structure and Mechanosensing

Schematic representation of effects of Skeletal Dysplasia mutations on FLN. In summary our results reveal that shear forces break a tight interaction between domains 16 and 17. Dysplasia associated mutations affect the structure of domains 16-17 either by destabilizing the internal structure of domain 17 or transforming 16-17 fragment from compact to an elongated form.