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Article

Inactivation of Invs/Nphp2 in renal epithelial cells drives infantile nephronophthisis like phenotypes in mouse.

eLife - 15 Mar 2023

Li Yuanyuan, Xu Wenyan, Makova Svetlana, Brueckner Martina, Sun Zhaoxia

Abstract excerpt

Nephronophthisis (NPHP) is a ciliopathy characterized by renal fibrosis and cyst formation, and accounts for a significant portion of end stage renal disease in children and young adults. Currently, no targeted therapy is available for this disease. INVS/NPHP2 is one of the over 25 NPHP genes identified to date. In mouse, global knockout of Invs leads to renal fibrosis and cysts. However, the precise contribution...

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Identifiers

PMID: 36920028
DOI: 10.7554/eLife.82395
PMCID: PMC10154023

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Inactivation of Invs/Nphp2 in renal epithelial cells drives infantile nephronophthisis like phenotypes in mouse.PMID 36920028Read ↗

Inactivation of <i>Nphp2</i> in renal epithelial cells drives infantile nephronophthisis like phenotypes in mouseSemantic proximity Read ↗

Renal Tubule-Specific Deletion of Nephrocystin 3 (Nphp3) Causes Infantile Nephronophthisis-like Phenotypes in Mice.Semantic proximity Read ↗

Developmental switch dichotomizes kidney response to <i>Nphp3</i> inactivation and treatment outcomeSemantic proximity Read ↗

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Six2creFrs2α knockout mice are a novel model of renal cystogenesis.Semantic proximity Read ↗

Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination.Semantic proximity Read ↗

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