ArticleJournal of the American Society of Nephrology : JASN2018
Activated Hedgehog-GLI Signaling Causes Congenital Ureteropelvic Junction Obstruction.
Article in Journal of the American Society of Nephrology : JASN, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 28 citations in OpenAlex.
- A systematic review of underlying genetic factors associated with ureteropelvic junction obstruction in stenotic human tissue.Journal of pediatric urology · 2022Pooled it
- Impaired intestinal calcium absorption and osteopathy in ICR/Mlac-hydro mice with hypoparathyroidism and severe hydronephrosis.Scientific reports · 2025Article
- Hedgehog signaling is required for the maintenance of mesenchymal nephron progenitors.bioRxiv : the preprint server for biology · 2024Article
- Hedgehog-GLI mediated control of renal formation and malformation.Frontiers in nephrology · 2023Review
- Expression and clinical implications of PARs in the stenotic tissue of ureteropelvic junction obstruction.Frontiers in pediatrics · 2023Article
- Understanding ureteropelvic junction obstruction: how far have we come?Frontiers in urology · 2023Review
- Evaluation of PGP 9.5, NGFR, TGFβ1, FGFR1, MMP-2, AT2R2, SHH, and TUNEL in Primary Obstructive Megaureter Tissue.The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society · 2022Article
- Transforming growth factor beta signaling functions during mammalian kidney development.Pediatric nephrology (Berlin, Germany) · 2021Review
- The genetic background significantly impacts the severity of kidney cystic disease in the Pkd1Kidney international · 2021Article
- Hedgehog Signaling: Implications in Cancers and Viral Infections.International journal of molecular sciences · 2021Review
- Roles for urothelium in normal and aberrant urinary tract development.Nature reviews. Urology · 2020Review
- Lineage-specific roles of hedgehog-GLI signaling during mammalian kidney development.Pediatric nephrology (Berlin, Germany) · 2020Review
- Overactivity or blockade of transforming growth factor-β each generate a specific ureter malformation.The Journal of pathology · 2019Article
Corrections and comments
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Authors and funding
11 authors at 7 institutions in 3 countries.
Funding
Abstract
Intrinsic ureteropelvic junction obstruction is the most common cause of congenital hydronephrosis, yet the underlying pathogenesis is undefined. Hedgehog proteins control morphogenesis by promoting GLI-dependent transcriptional activation and inhibiting the formation of the GLI3 transcriptional repressor. Hedgehog regulates differentiation and proliferation of ureteric smooth muscle progenitor cells during murine kidney-ureter development. Histopathologic findings of smooth muscle cell hypertrophy and stroma-like cells, consistently observed in obstructing tissue at the time of surgical correction, suggest that Hedgehog signaling is abnormally regulated during the genesis of congenital intrinsic ureteropelvic junction obstruction. Here, we demonstrate that constitutively active Hedgehog signaling in murine intermediate mesoderm-derived renal progenitors results in hydronephrosis and failure to develop a patent pelvic-ureteric junction. Tissue obstructing the ureteropelvic junction was marked as early as E13.5 by an ectopic population of cells expressing
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