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Impaired Wnt-beta-catenin signaling disrupts adult renal homeostasis and leads to cystic kidney ciliopathy.

Nature medicine | Oct 2, 2009

Cystic kidney disease represents a major cause of end-stage renal disease, yet the molecular mechanisms of pathogenesis remain largely unclear. Recent emphasis has been placed on a potential role for canonical Wnt signaling, but investigation of this pathway in adult renal homeostasis is lacking. Here we provide evidence of a previously unidentified canonical Wnt activity in adult mammalian kidney homeostasis, the loss of which leads to cystic kidney disease. Loss of the Jouberin (Jbn) protein in mouse leads to the cystic kidney disease nephronophthisis, owing to an unexpected decrease in endogenous Wnt activity. Jbn interacts with and facilitates beta-catenin nuclear accumulation, resulting in positive modulation of downstream transcription. Finally, we show that Jbn is required in vivo for a Wnt response to injury and renal tubule repair, the absence of which triggers cystogenesis.

Pubmed ID: 19718039 RIS Download

Mesh terms: Animals | Cilia | Homeostasis | Kidney | Kidney Diseases, Cystic | Mice | Mice, Knockout | Proto-Oncogene Proteins | Signal Transduction | Wnt Proteins | beta Catenin

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Associated grants

  • Agency: NINDS NIH HHS, Id: P30 NS047101-05
  • Agency: NINDS NIH HHS, Id: P30 NS047101
  • Agency: NINDS NIH HHS, Id: R01 NS048453
  • Agency: NIGMS NIH HHS, Id: T32 GM08666
  • Agency: NINDS NIH HHS, Id: P30 NS047101-06A1
  • Agency: NINDS NIH HHS, Id: P30 NS047101-03
  • Agency: NINDS NIH HHS, Id: R01 NS052455
  • Agency: NINDS NIH HHS, Id: P30 NS047101-03S1
  • Agency: NINDS NIH HHS, Id: R01 NS052455-03
  • Agency: NIGMS NIH HHS, Id: T32 GM008666
  • Agency: NINDS NIH HHS, Id: P30 NS047101-04

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