Cilia-dependent inflammatory signaling in homeostasis and response to injury

My research focuses on how primary cilia, a tiny antenna-like structure popping-up at the apical surface of kidney cells, control kidney homeostasis and contribute to disease. I have shown that mutations in ciliary genes responsible for nephronophthisis and polycystic kidney disease trigger abnormal inflammatory responses that drive kidney scarring. More broadly, I have established that the primary cilium acts as a signalling platform governing communication between tubular epithelial cells and immune cells. My current work explores how cilia sense danger signals released during kidney infection or injury, and how defects in this sensing mechanism underlie renal ciliopathies - with the ultimate goal of identifying new therapeutic targets for these rare, currently incurable diseases.

Trained as a geneticist at the “Magistère Européen de Génétique”, I moved to pathophysiology through a PhD training in Dr Terzi team (Paris, France) obtained in 2009. I gained expertise in modelling kidney ciliopathies in Pr Kuehn team (Nephrolab, Freiburg, Germany). In 2019, I joined Dr Saunier lab (LMRH, Paris, France) to pursue my research on cilia functions in the regulation of kidney inflammation, focusing on renal ciliopathies. In 2022, I was appointed as an INSERM researcher (CRCN) and obtained my HDR in 2023. I was appointed as Principal Investigator by the scientific advisory board of Imagine in 2026.

Selected publications

• Cilia to basement membrane signaling is a biomechanical driver in models of autosomal dominant polycystic kidney disease. (2026). Manal Mazloum, Brice Lapin, Rushdi Alghamdi, Jessica Vandensteen, Martine Burtin, Pascal Houillier, Lydie Cheval, Gilles Crambert, Vicky Scata, Camille Cohen, Christoph Schell, Michael Rehman, Amandine Aka, Karim Ourahmoun, Rui Benedito, E. Wolfgang Kuehn, Stéphanie Descroix, Tilman Busch, Michael Köttgen, Serge Garbay, Marie-Christine Verpont, Ellie Tang, Brigitte Lelongt, Nicolas Cagnard, Stefan Somlo, Sylvie Coscoy, Fabiola Terzi, Amandine Viau, and Frank Bienaimé. J Clin Invest. 
• Developmental switch dichotomizes kidney response to Nphp3 inactivation and treatment outcome. (2026). Joran Martin^$, Alice S Serafin^$, Florine Chereau, Younes Achouri, Nicolas Cagnard, Marie-Christine Verpont, Alexandre Benmerah, Isabelle Scheers, Patrick Jacquemin, Sophie Saunier, Amandine Viau. bioRxiv. doi.org/10.64898/2026.05.21.726570. ^$Co-first authors

• A cilia-dependent inflammatory programme links bacterial detection to kidney disease. (2026). Amandine Aka^$, Joran Martin^$, Fanny Kopp, Teresa Bulfone, Samuel Chauvin, Camille Cohen, Martine Burtin, E. Wolfgang Kuehn, Anne-Sophie Dangeard, Maryse Moya-Nilges, Nicolas Cagnard, Nicolas Goudin, Meriem Garfa-Traoré, Marceau Quatredeniers, Cong Xu, Laurent Audry, Jost Enninga, Matthieu Rousseau, Cécile Arrieumerlou, Jieqing Fan, Sophie Saunier, Fabiola Terzi, Molly A. Ingersoll, Henri Lichenstein, Amandine Viau*, Frank Bienaimé*. bioRxiv. ^$Co-first authors, * co-last authors.

• Inhibition of the inflammasome ameliorates orthologous polycystic kidney disease. (2025). Liu J, Rogg M, Moos K, Sasanpour S, Strassl V, Jain M, Magassa S, Neubauer B, Braeg S, Saller B.S, Weisser L, Bienaimé B, Gorka O, Boerries M, Viau A, Gross O, Schell C, Kuehn E.W. Proc Natl Acad Sci. 

• IL1b is induced in nephronophthisis but does not mediate kidney damage. (2025). Ferri G, El Hamdaoui M, Martin M, Kuehn E.W, Bienaimé F, Saunier S, Viau A. Genes & Diseases. doi.org/10.1016/j.gendis.2025.101687

• The ANKS3/BICC1 protein complex is a master post-transcriptional regulator of NPHP1 ciliopahy-gene transcripts. (2024). Mahuzier A, Odye G, Grampa V, Bizet A, Viau A, Ryan R, Mehraz M, Blisnick T, Clark E, Henry C, Piedagnel R, Silbermann F, Hayot G, De Grande L, Kahn A, Plaza JM, Krug P, Knebelmann B, Muller F, Bastin P, Sailer A. W, Saint-Mezard P, Jeanpierre C, Hoffmann S, Benmerah A, Lelongt B, Delous M, Saunier S. bioRxiv. doi.org/10.1101/2024.04.18.588747.

• Meta-analysis of single-cell and single-nucleus transcriptomics reveals kidney cell type consensus signatures. (2023). Quatredeniers M, Serafin A, Benmerah A, Raussell A, Saunier S, Viau A. Sci Data. 

• YAP restricts renal inflammation and mitigates kidney damage in nephronopthisis related kidney disease. (2022). Ferri G, Goffette V, Porée E, Braeg S, Carvalho S, Quatredeniers M, Kuehn E.W, Saunier S, Viau A. bioRxiv. 

• Agonists of prostaglandin E2 receptors as potential first in class treatment for nephronophthisis and related ciliopathies. (2022). Garcia H*, Serafin A*, Silbermann F*, Porée E, Viau A, Mahaut C, Billot K, Birgy E, Garfa-Traore M, Roy S, Cecarelli S, Mehraz M, Rodriguez P-C, Deleglise B, Furio L, Jabot-Hanin F, Cagnard N, Del Nery E, Fila M, Sin-Monnot S, Antignac C, Lyonnet S, Krug P, Salomon R, Annereau J-P, Benmerah A, Delous M, Briseo-Roa L, Saunier S. Proc Natl Acad Sci. 

• The renal inflammatory network of nephronophthisis. (2022). Quatredeniers M, Bienaimé F, Ferri G, Isnard P, Porée E, Billot K, Birgy E, Ceccarelli S, Legendre F, Braeg S, Nguyen-Khoa T, Salomon R, Gubler M-C, Kühn E.W, Saunier S, Viau A. Hum. Mol. Genet. 

• Tubular STAT3 limits renal inflammation in autosomal dominant polycystic kidney disease. (2020) Viau A, Baaziz M, Aka A, Nguyen C, Kuehn E.W, Terzi F, Bienaimé F.  J Am Soc Nephrol. 

• Cilia‐localized LKB1 regulates chemokine signaling, macrophage recruitment, and tissue homeostasis in the kidney. (2018). Viau A*, Bienaimé F*, Lukas K, Todkar A.P, Knoll M, Yakulov T.A, Hofherr A, Kretz O, Helmstädter M, Reichardt W, Braeg S, AschmanT, Merkle A, Pfeifer D, Dumit V.I, Gubler MC, Nitschke R, Huber T.B, Terzi F, Dengjel J, Grahammer F, Köttgen M, Busch H, Boerries M, Walz G, Triantafyllopoulou A, Kuehn E.W. EMBO J. 

Team members

Dr Valentine Goffette, Postdoc
Joël Kangah, AI
Adam Maatoug, M2 student
Joran Martin, PhD
 

Alumni
Florine Chereau, IE & M2 student
Line Tlemçani, M2 student
Giulia Ferri, PhD
Fatou Keita, M1 student
Mariyam El Hamdaoui, Engineer student
Salomé Ceccarelli, Engineer student
Shana Carvalho, BTS student