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YAP restricts renal inflammation and mitigates kidney damage in nephronothisis related kidney disease Open

Giulia Ferri, Valentine Goffette, Esther Porée, Simone Braeg, Shana Carvalho , et al. · 2022

Biology Medicine

Nephronophthisis (NPH) is an orphan recessive kidney disease mostly caused by mutations in NPHP1 and 20 other genes encoding proteins that localize to primary cilia. To date the pathways linking altered primary cilia function to progressiv…

The renal inflammatory network of nephronophthisis Open

Marceau Quatredeniers, Frank Bienaimé, Giulia Ferri, Pierre Isnard, Esther Porée , et al. · 2022

Biology

Renal ciliopathies are the leading cause of inherited kidney failure. In autosomal dominant polycystic kidney disease (ADPKD), mutations in the ciliary gene PKD1 lead to the induction of CCL2, which promotes macrophage infiltration in the …

The renal inflammatory network of nephronophthisis Open

Marceau Quatredeniers, Frank Bienaimé, Giulia Ferri, Pierre Isnard, Esther Porée , et al. · 2021

Medicine Biology

STRUCTURED ABSTRACT BACKGROUND The majority of genetic kidney disease leading to kidney failure is caused by mutations in ciliary genes. How cilia malfunction leads to progressive kidney damage is poorly understood, but recent evidence lin…

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