The Ciliary Protein Nephrocystin-4 Translocates the Canonical Wnt Regulator Jade-1 to the Nucleus to Negatively Regulate β-Catenin Signaling

Background: Deregulation of Wnt signaling contributes to the development of cystic kidney diseases such as nephronophthisis (NPH).Results: The NPH protein NPHP4 stabilizes Jade-1, a negative Wnt regulator, and translocates Jade-1 to the nucleus.Conclusion: NPHP4 and Jade-1 additively decrease canonical Wnt signaling.Significance: Loss of NPHP4-mediated Wnt repression via Jade-1 may contribute to cystogenesis in NPH.