RPGRIP1L and ciliopathy: We assume that the reduced TZ amount of Cep290 in the absence of Rpgrip1l might be very much involved in the development of the ciliopathy phenotype of Rpgrip1l−/− mouse embryos but since Cep290 deficiency shortened cilia in NIH3T3 cells (Fig 3G), while the loss of Rpgrip1l elongated cilia in MEFs (Gerhardt et al, 2015), it is unlikely that the decreased Cep290 TZ amount is solely responsible for the severe ciliopathy caused by Rpgrip1l deficiency.