To address these questions, it will be necessary to examine the TZ assembly in various (prenatal and postnatal) cell types of patients suffering from ciliopathies caused by mutations in RPGRIP1L. These future investigations are essential, because our data illustrate the consequences caused by a complete loss of Rpgrip1l, while most patients have hypomorphic mutations in RPGRIP1L. The understanding of the regulation of TZ assembling in humans is a promising research subject to pave the way for the development of therapies against ciliopathies. The gene discussed is RPGRIP1L; the disease is ciliopathy.