Inversin relays Frizzled-8 signals to promote proximal pronephros development

08.11.2010

Lienkamp S, Ganner A, Boehlke C, Schmidt T, Arnold SJ, Schäfer T, Romaker D, Schuler J, Hoff S, Powelske C, Eifler A, Krönig C, Bullerkotte A, Nitschke R, Kuehn EW, Kim E, Burkhardt H, Brox T, Ronneberger O, Gloy J, Walz G.

Proc Natl Acad Sci U S A. 107(47):20388-93

Proc Natl Acad Sci U S A      online article

Mutations of inversin cause type II nephronophthisis, a hereditary disease characterized by cystic kidneys and developmental defects. We show that Inversin is essential for Xenopus pronephros morphogenesis, involving two distinct forms of cell movements. Knockdown of Inversin abrogated proximal pronephros extension downstream of Frizzled-8. Depletion of Inversin impedes the accumulation of Dishevelled at the apical membrane of tubule cells in vivo. Thus, defective tubule morphogenesis may contribute to the renal pathology of patients with nephronophthisis type II.