Click here to close Hello! We notice that you are using Internet Explorer, which is not supported by Xenbase and may cause the site to display incorrectly. We suggest using a current version of Chrome, FireFox, or Safari.
XB-ART-1988
Nat Genet 2005 May 01;375:537-43. doi: 10.1038/ng1552.
Show Gene links Show Anatomy links

Inversin, the gene product mutated in nephronophthisis type II, functions as a molecular switch between Wnt signaling pathways.

Simons M , Gloy J , Ganner A , Bullerkotte A , Bashkurov M , Krönig C , Schermer B , Benzing T , Cabello OA , Jenny A , Mlodzik M , Polok B , Driever W , Obara T , Walz G .


???displayArticle.abstract???
Cystic renal diseases are caused by mutations of proteins that share a unique subcellular localization: the primary cilium of tubular epithelial cells. Mutations of the ciliary protein inversin cause nephronophthisis type II, an autosomal recessive cystic kidney disease characterized by extensive renal cysts, situs inversus and renal failure. Here we report that inversin acts as a molecular switch between different Wnt signaling cascades. Inversin inhibits the canonical Wnt pathway by targeting cytoplasmic dishevelled (Dsh or Dvl1) for degradation; concomitantly, it is required for convergent extension movements in gastrulating Xenopus laevis embryos and elongation of animal cap explants, both regulated by noncanonical Wnt signaling. In zebrafish, the structurally related switch molecule diversin ameliorates renal cysts caused by the depletion of inversin, implying that an inhibition of canonical Wnt signaling is required for normal renal development. Fluid flow increases inversin levels in ciliated tubular epithelial cells and seems to regulate this crucial switch between Wnt signaling pathways during renal development.

???displayArticle.pubmedLink??? 15852005
???displayArticle.pmcLink??? PMC3733333
???displayArticle.link??? Nat Genet
???displayArticle.grants??? [+]

Species referenced: Xenopus laevis
Genes referenced: ankrd6 dvl2 invs
GO keywords: non-canonical Wnt signaling pathway [+]
???displayArticle.morpholinos??? invs MO1

???displayArticle.disOnts??? physical disorder [+]
???displayArticle.omims??? NEPHRONOPHTHISIS 2; NPHP2
References [+] :
Das, Diego interacts with Prickle and Strabismus/Van Gogh to localize planar cell polarity complexes. 2004, Pubmed