Review Article

The Role of RPGR and Its Interacting Proteins in Ciliopathies

Figure 4

RPGR interacting protein network. Black arrows show direct interaction; the big blue arrow shows proteins in RPGR complex detected by coimmunoprecipitation. Proteins labelled in pink are implicated in retinal degeneration. The RCC1-like domain directly interacts with RPGRIP1, RPGRIP1L, SMC1/3, PDEδ, and Rab8. The end section of the C-terminal directly interacts with nucleophosmin and whirlin. Mutations in RPGRIP1, RPGRIP1L, CEP290, NPHP4, NPHP5, IFT88, KIF3A, and SPATA7 also cause ciliopathies.