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XB-ART-42213
Proc Natl Acad Sci U S A November 23, 2010; 107 (47): 20388-93.
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Inversin relays Frizzled-8 signals to promote proximal pronephros development.

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 .


Abstract
Mutations of inversin cause type II nephronophthisis, an infantile autosomal recessive disease characterized by cystic kidney disease and developmental defects. Inversin regulates Wnt signaling and is required for convergent extension movements during early embryogenesis. We now show that Inversin is essential for Xenopus pronephros formation, involving two distinct and opposing forms of cell movements. Knockdown of Inversin abrogated both proximal pronephros extension and distal tubule differentiation, phenotypes similar to that of Xenopus deficient in Frizzled-8. Exogenous Inversin rescued the pronephric defects caused by lack of Frizzled-8, indicating that Inversin acts downstream of Frizzled-8 in pronephros morphogenesis. Depletion of Inversin prevents the recruitment of Dishevelled in response to Frizzled-8 and impeded the accumulation of Dishevelled at the apical membrane of tubular epithelial cells in vivo. Thus, defective tubule morphogenesis seems to contribute to the renal pathology observed in patients with nephronophthisis type II.

PubMed ID: 21059920
PMC ID: PMC2996658
Article link: Proc Natl Acad Sci U S A


Species referenced: Xenopus laevis
Genes referenced: atp1a1 axin2 clcnkb dvl1 dvl3 fn1 fzd8 gata3 hnf1b invs lhx1 nphs1 pax8 slc12a1 slc12a3 slc4a4 slc5a1.2
GO keywords: pronephric nephron tubule morphogenesis
Antibodies: Kidney Ab1 Kidney Ab2
Morpholinos: dvl1 MO1 dvl3 MO1 fzd8 MO1 invs MO1

Disease Ontology terms: nephronophthisis 2
OMIMs: NEPHRONOPHTHISIS 2; NPHP2
Phenotypes: Xla Wt + DN-dvl1 (Fig. S 6 F) [+]

Article Images: [+] show captions
References [+] :
Ando, An optical marker based on the UV-induced green-to-red photoconversion of a fluorescent protein. 2002, Pubmed