The RCC1-like region also interacts with: RPGR interaction protein 1 (RPGRIP1), which links it to the connecting cilium of photoreceptor cells [19]; the lipid trafficking protein phosphodiesterase 6D (PDE6D) [22]; two chromosome-associated proteins important for the structural maintenance of chromosomes, SMC1 and SMC3 [23] and two ciliary disease-associated proteins nephrocystin-5 (NPHP5) [24] and centrosomal protein 290 (CEP290) [25]. The gene discussed is RPGRIP1; the disease is glycogen storage disease VI.