It is tempting to speculate that downregulation of RNF8 not only underlies the defective repair connected with loss of splicing-related proteins, but is also involved in human diseases such as retinitis pigmentosa, which is caused by inherited mutations in the gene encoding the splicing factor PRPF8 and associated with severe vision impairment and even blindness.31, 32 Our discovery that PRPF8 regulates RNF8-mediated DNA repair not only reveals a novel function of the PRPF8 protein, but also improves our understanding of the molecular mechanisms underlying this disease. Here, RNF8 is linked to retinitis pigmentosa.