RPGR and ciliopathy: These observations are further supported by other studies whereby meckelin (MKS3), through interaction with Nesprin-2, also regulates the actin cytoskeleton (39,40); loss of function of the ciliopathy protein, TMEM216 leads to defective ciliogenesis and centrosomal docking, with concomitant hyperactivation of RhoA and Dishevelled (41); and RPGR-deficient cells have reduced numbers of cilia, slower cell cycle progression, impaired substrate attachment and increased FA stability (16).