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. 2019 Aug 31;117(8):1467–1475. doi: 10.1016/j.bpj.2019.08.032

Figure 1.

Figure 1

An overview of the structural organization of FLNA. (A) Shown is the schematic representation of FLNA dimer having 24 Ig domains (gray) and the actin-binding domain (ABD) (yellow) in a monomer. The two monomers are dimerized via domain 24. Rod1 consists of domains 1–15, and rod2 consists of domains 16–23. The studied domains, 4–6, are colored in pale purple, pale green, and blue, respectively. The localization of FLNA-MVD P637Q mutation in domain 4 and V711D and H743P mutations in domain 5 have been highlighted with stars. The fourth missense mutation causing FLNA-MVD, G288R, is located in domain 1. (B) The crystal structure of FLNA3–5 (PDB: 4M9P) (14) is colored in pink (FLNA3), pale purple (FLNA4), and pale green (FLNA5). Residues V711 and H743, whose mutation to aspartic acid and proline residues, respectively, cause FLNA-MVD, are shown with ball-and-stick models. To see this figure in color, go online.