ArticleJournal of the American Society of Nephrology : JASN2012
Sprouty1 haploinsufficiency prevents renal agenesis in a model of Fraser syndrome.
Article in Journal of the American Society of Nephrology : JASN, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 26 citations in OpenAlex.
- The term CAKUT has outlived its usefulness: the case for the prosecution.Pediatric nephrology (Berlin, Germany) · 2022Review
- Nephron Progenitor Maintenance Is Controlled through Fibroblast Growth Factors and Sprouty1 Interaction.Journal of the American Society of Nephrology : JASN · 2020Article
- Liver Buds and Liver Organoids: New Tools for Liver Development, Disease and Medical Application.Stem cell reviews and reports · 2019Review
- Overactivity or blockade of transforming growth factor-β each generate a specific ureter malformation.The Journal of pathology · 2019Article
- Renal development in the fetus and premature infant.Seminars in fetal & neonatal medicine · 2017Review
- Pharyngeal morphogenesis requires fras1-itga8-dependent epithelial-mesenchymal interaction.Developmental biology · 2016Article
- Fibroblast growth factor receptor signaling in kidney and lower urinary tract development.Pediatric nephrology (Berlin, Germany) · 2016Review
- Syndactyly in a novel Fras1(rdf) mutant results from interruption of signals for interdigital apoptosis.Developmental dynamics : an official publication of the American Association of Anatomists · 2016Article
- From gene discovery to new biological mechanisms: heparanases and congenital urinary bladder disease.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2016Review
- Mild recessive mutations in six Fraser syndrome-related genes cause isolated congenital anomalies of the kidney and urinary tract.Journal of the American Society of Nephrology : JASN · 2014Article
- Novel frem1-related mouse phenotypes and evidence of genetic interactions with gata4 and slit3.PloS one · 2013Article
- Cytokeratin 15 marks basal epithelia in developing ureters and is upregulated in a subset of urothelial cell carcinomas.PloS one · 2013Article
- GDNF-independent ureteric budding: role of PI3K-independent activation of AKT and FOSB/JUN/AP-1 signaling.Biology open · 2013Article
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Authors and funding
4 authors at 3 institutions in 2 countries.
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Abstract
Deficiency of the extracellular matrix molecule FRAS1, normally expressed by the ureteric bud, leads to bilateral renal agenesis in humans with Fraser syndrome and blebbed (Fras1(bl/bl)) mice. The metanephric mesenchyme of these mutants fails to express sufficient Gdnf, which activates receptor tyrosine kinase (RTK) signalling, contributing to the phenotype. To determine whether modulating RTK signalling may overcome the abnormal nephrogenesis characteristic of Fraser syndrome, we introduced a single null Sprouty1 allele into Fras1(bl/bl) mice, thereby reducing the ureteric bud's expression of this anti-branching molecule and antagonist of RTK signalling. This prevented renal agenesis in Fras1(bl/bl) mice, permitting kidney development and postnatal survival. We found that fibroblast growth factor (FGF) signalling contributed to this genetic rescue, and exogenous FGF10 rescued defects in Fras1(bl/bl) rudiments in vitro. Whereas wild-type metanephroi expressed FRAS1 and the related proteins FREM1 and FREM2, FRAS1 was absent and the other proteins were downregulated in rescued kidneys, consistent with a reciprocally stabilized FRAS1/FREM1/FREM2 complex. In addition to contributing to knowledge regarding events during nephrogenesis, the demonstrated rescue of renal agenesis in a model of a human genetic disease raises the possibility that enhancing growth factor signaling might be a therapeutic approach to ameliorate this devastating malformation.
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