Knockout of Ror2 and Wnt5a exhibited phenotypes similar to those found in RS patients.16,19, -21 Hypoplastic phalanges in Ror2−/− embryos were also shown as compared with the wild type, reflecting the mild brachydactyly seen in some ARRS patients.22 Wnt5a was also found to be necessary for kidney development in both mouse and zebrafish phenocopied to RS patients.5,15,16,23,24 Moreover, the NXN gene encodes nucleoredoxin, a regulator of the Wnt/PCP pathway through binding with DVL. This evidence concerns the gene ROR2 and X-linked retinoschisis.