To reveal the molecular processes underlying ciliopathies caused by mutations in RPGRIP1L, future studies should clarify to what extent the disturbances in TZ assembling and of the degradation systems contribute to the ciliopathy of Rpgrip1l−/− mouse embryos, whether proteasomal activity at the ciliary base and/or autophagic activity are affected in patients suffering from ciliopathies caused by mutations in RPGRIP1L and if there are interactions between TZ assembling and the degradation systems which ensure the proper development and homeostasis in vertebrates. The gene discussed is RPGRIP1L; the disease is ciliopathy.