Impaired Wnt–β-catenin signaling disrupts adult renal homeostasis and leads to cystic kidney ciliopathy
Open accessAbstract
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.
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Ciliary JBN facilitates ciliary localization of beta-catenin following WNT3A stimulation and negatively affects WNT signalling.
“Ciliary JBN facilitates the ciliary localization of β-catenin following WNT3A stimulation and thereby negatively affects WNT signalling 206.”
From Cellular signalling by primary cilia in development, organ function and disease (2019) · cites this paper as [206]
In Results, ‘Jbn facilitates β-catenin nuclear translocation’ (page unavailable), the paper reports that Jbn enhances Wnt3a-dependent β-catenin accumulation in the nucleus, not the cilium. In Results, ‘Jbn functions downstream of β-catenin stabilization’ (page unavailable), it reports that Jbn potentiates the response to Wnt3a and positively modulates canonical Wnt signaling, contrary to the claimed negative effect. (LLM, conf=0.90)
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References 57 · Cited by 175
Research works printed but not linked: 1
- [15]Davison AM Oxford Textbook of Clinical Nephrology Section 16.3 Oxford University Press Oxford 2005
- Authors
- Lancaster, M. A., Louie, C. M., Silhavy, J. L., Sintasath, L., DeCambre, M., Nigam, S. K., Willert, K., & Gleeson, J. G.
- Affiliations
- University of California San Diego (US)
- Howard Hughes Medical Institute (US)
- Corresponding author
- Gleeson, Joseph G
- Venue
- Nature Medicine
- Published
- 2009-08-30
- Volume
- 15
- Issue
- 9
- Pages
- 1046-1054
- ISSN
- 1078-8956 (ISSN-L)
- 1546-170X
- PMC
- PMC2895985
- OpenAlex
- W1998590298
- Cited by
- 175
- References
- 57
- Citations by year
- 2026: 2 · 2025: 5 · 2024: 3 · 2023: 6 · 2022: 4 · 2021: 9 · 2020: 10 · 2019: 6 · 2018: 10 · 2017: 7 · 2016: 6 · 2015: 11 · 2014: 13 · 2013: 14 · 2012: 21
- FWCI
- 13.38
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Medicine (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institute of General Medical Sciences (T32GM08666, T32GM008666)
- National Institute of Neurological Disorders and Stroke (R01NS048453, R01NS052455, P30NS047101)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Wnt/β-catenin signaling in development and cancer
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Renal and related cancers
- Keywords
- Wnt/β-catenin signaling, β-catenin, renal homeostasis, cystic kidney disease, nephronophthisis, kidney injury, cyst formation, end-stage renal disease
- MeSH terms
- Animals, Cilia, Homeostasis, Kidney, Proto-Oncogene Proteins, Signal Transduction, Mice, Knockout, Adaptor Proteins, Vesicular Transport, Wnt Proteins, beta Catenin, Mice, Kidney Diseases, Cystic
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