This analysis demonstrates that both SIX1 and SIX1-Q177R recapitulate key NPC regulatory activities that could contribute to the persistence of NPC-like characteristics in Wilms tumor, and that SIX1-Q177R is a candidate for regulation of several differentially expressed genes in SIX1/2-Q177R tumors. The gene discussed is SIX1; the disease is Nephroblastoma.