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Affichage: 16 résultats.
    • Article de périodique
    Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiency
    Luciani, Alessandro Schumann, Anke Berquez, Marine Chen, Zhiyong Nieri, Daniela Failli, Mario Debaix, Huguette Festa, Beatrice Paola Tokonami, Natsuko Raimondi, Andrea Cremonesi, Alessio Carrella, Diego Forny, Patrick Kölker, Stefan Diomedi Camassei, Francesca Diaz, Francisca Moraes, Carlos T. Di Bernardo, Diego Baumgartner, Matthias R. Devuyst, Olivier[UCL] (2020) Nature Communications — Vol. 11, no. 1, p. 970 [1-21] (2020)
    • Article de périodique
    Bone marrow transplantation improves proximal tubule dysfunction in a mouse model of Dent disease
    Gabriel, Sarah S. Belge, Hendrica Gassama, Alkaly Debaix, Huguette Luciani, Alessandro Fehr, Thomas Devuyst, Olivier[UCL] (2017) Kidney International — Vol. 91, no.4, p. 842-855 (2017)
    • Article de périodique
    Cell-Based Phenotypic Drug Screening Identifies Luteolin as Candidate Therapeutic for Nephropathic Cystinosis
    De Leo, Ester Elmonem, Mohamed A. Berlingerio, Sante Princiero Berquez, Marine Festa, Beatrice Paola Raso, Roberto Bellomo, Francesco Starborg, Tobias Janssen, Manoe Jacoba Abbaszadeh, Zeinab Cairoli, Sara Goffredo, Bianca Maria Masereeuw, Rosalinde Devuyst, Olivier[UCL] Lowe, Martin Levtchenko, Elena Luciani, Alessandro Emma, Francesco Rega, Laura Rita (2020) Journal of the American Society of Nephrology — Vol. 31, no. 7, p. 1522-1537 (2020)
    • Article de périodique
    Receptor-Mediated Endocytosis and Differentiation in Proximal Tubule Cell Systems
    Berquez, Marine Krohn, Patrick Luciani, Alessandro Devuyst, Olivier[UCL] (2021) Journal of the American Society of Nephrology — Vol. 32, no.5, p. 1265-1267 (2021)
    • Article de périodique
    Author Correction: Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiency
    Luciani, Alessandro Schumann, Anke Berquez, Marine Chen, Zhiyong Nieri, Daniela Failli, Mario Debaix, Huguette Festa, Beatrice Paola Tokonami, Natsuko Raimondi, Andrea Cremonesi, Alessio Carrella, Diego Forny, Patrick Kölker, Stefan Camassei, Francesca Diomedi Diaz, Francisca Moraes, Carlos T. Di Bernardo, Diego Baumgartner, Matthias R. Devuyst, Olivier[UCL] (2020) Nature Communications — Vol. 11, no. 1, p. 1719 (2020)
    • Article de périodique
    Defective autophagy degradation and abnormal tight junction-associated signaling drive epithelial dysfunction in cystinosis
    Luciani, Alessandro Festa, Beatrice Paola Chen, Zhiyong Devuyst, Olivier[UCL] (2018) Autophagy — Vol. 14, no.7, p. 1157-1159 (2018)
    • Article de périodique
    The phosphoinositide 3-kinase inhibitor alpelisib restores actin organization and improves proximal tubule dysfunction in vitro and in a mouse model of Lowe syndrome and Dent disease
    Berquez, Marine Gadsby, Jonathan R. Festa, Beatrice Paola Butler, Richard Jackson, Stephen P. Berno, Valeria Luciani, Alessandro Devuyst, Olivier[UCL] Gallop, Jennifer L. (2020) Kidney International — Vol. 98, no. 4, p. 883-896 (2020)
    • Article de périodique
    OCRL deficiency impairs endolysosomal function in a humanized mouse model for Lowe syndrome and Dent disease
    Festa, Beatrice Paola Berquez, Marine Gassama, Alkaly Amrein, Irmgard Ismail, Hesham M Samardzija, Marijana Staiano, Leopoldo Luciani, Alessandro Grimm, Christian Nussbaum, Robert L De Matteis, Maria Antonietta Dorchies, Olivier M Scapozza, Leonardo Wolfer, David Paul Devuyst, Olivier[UCL] (2018) Human Molecular Genetics — Vol. 28, no.12, p. 1931-1946 (2018)
    • Article de périodique
    Transgenic zebrafish modeling low-molecular-weight proteinuria and lysosomal storage diseases
    Chen, Zhiyong Luciani, Alessandro Mateos, José María Barmettler, Gery Giles, Rachel H. Neuhauss, Stephan C.F. Devuyst, Olivier[UCL] (2020) Kidney International — Vol. 97, no. 6, p. 1150-1163 (2020)
    • Article de périodique
    Methylmalonyl acidemia: from mitochondrial metabolism to defective mitophagy and disease
    Luciani, Alessandro[UCL] Devuyst, Olivier[UCL] (2020) Autophagy — Vol. 16, no. 6, p. 1159-1161 (2020)
    • Article de périodique
    Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
    Festa, Beatrice Paola Chen, Zhiyong Berquez, Marine Debaix, Huguette Tokonami, Natsuko Prange, Jenny Ann Hoek, Glenn van de Alessio, Cremonesi Raimondi, Andrea Nevo, Nathalie Giles, Rachel H. Devuyst, Olivier[UCL] Luciani, Alessandro (2018) Nature Communications — Vol. 9, p. 161 [1-17] (2018)
    • Article de périodique
    Dedifferentiation and aberrations of the endolysosomal compartment characterize the early stage of nephropathic cystinosis.
    Raggi, Claudia[UCL] Luciani, Alessandro Nevo, Nathalie Antignac, Corinne Terryn, Sara[UCL] Devuyst, Olivier[UCL] (2014) Human Molecular Genetics — Vol. 23, no. 9, p. 2266-2278 (2014)
    • Article de périodique
    Multisystem involvement, defective lysosomes and impaired autophagy in a novel rat model of nephropathic cystinosis
    Krohn, Patrick Rega, Laura Rita Harvent, Marianne Festa, Beatrice Paola Taranta, Anna Luciani, Alessandro Dewulf, Joseph P.[UCL] Cremonesi, Alessio Camassei, Francesca Diomedi Hanson, James V M Gerth-Kahlert, Christina Emma, Francesco Berquez, Marine Devuyst, Olivier[UCL] (2022) Human molecular genetics — Vol. 31, no. 13, p. 2262-2278 (2022)
    • Article de périodique
    Autophagosome-lysosome fusion triggers a lysosomal response mediated by TLR9 and controlled by OCRL.
    De Leo, Maria Giovanna Staiano, Leopoldo Vicinanza, Mariella Luciani, Alessandro Carissimo, Annamaria Mutarelli, Margherita Di Campli, Antonella Polishchuk, Elena Di Tullio, Giuseppe Morra, Valentina Levtchenko, Elena Oltrabella, Francesca Starborg, Tobias Santoro, Michele di Bernardo, Diego Devuyst, Olivier[UCL] Lowe, Martin Medina, Diego L Ballabio, Andrea De Matteis, Maria Antonietta (2016) Nature Cell Biology — Vol. 18, no. 8, p. 839-850 (2016)
    • Article de périodique
    Impaired Lysosomal Function Underlies Monoclonal Light Chain-Associated Renal Fanconi Syndrome.
    Luciani, Alessandro Sirac, Christophe Terryn, Sara[UCL] Javaugue, Vincent Prange, Jenny Ann Bender, Sébastien Bonaud, Amélie Cogné, Michel Aucouturier, Pierre Ronco, Pierre Bridoux, Frank Devuyst, Olivier[UCL] (2015) Journal of the American Society of Nephrology — Vol. 27, p. 2049-2061 (2015)
    • Article de périodique
    Chloride transporters and receptor-mediated endocytosis in the renal proximal tubule
    Devuyst, Olivier[UCL] Luciani, Alessandro (2015) The Journal of Physiology — Vol. 593, no.18, p. 4151-4164 (2015)