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11 results on '"Ordovàs, Laura"'

2. Generation of a human induced pluripotent stem cell–based model for tauopathies combining three microtubule-associated protein TAU mutations which displays several phenotypes linked to neurodegeneration

Author

García-León, Juan Antonio, Cabrera-Socorro, Alfredo, Eggermont, Kristel, Swijsen, Ann, Terryn, Joke, Fazal, Raheem, Nami, FatemehArefeh, Ordovás, Laura, Quiles, Ana, Lluis, Frederic, Serneels, Lutgarde, Wierda, Keimpe, Sierksma, Annerieke, Kreir, Mohamed, Pestana, Francisco, Van Damme, Philip, De Strooper, Bart, Thorrez, Lieven, Ebneth, Andreas, and Verfaillie, Catherine M.

Published
2018
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9. Restoration of progranulin expression rescues cortical neuron generation in an induced pluripotent stem cell model of frontotemporal dementia

Author

Raitano, Susanna, Ordovàs, Laura, Geraerts, Martine, Khurana, Satish, Vanuytsel, Kim, Verfaillie, Catherine, De Muynck, Louis, Guo, Wenting, Vandenberghe, Rik, Van Den Bosch, Ludo, Van Damme, Philip, Espuny Camacho, Ira Mercedes, Vanderhaeghen, Pierre, Tóth, Balázs, Voets, Thomas, Cathomen, Toni, Raitano, Susanna, Ordovàs, Laura, Geraerts, Martine, Khurana, Satish, Vanuytsel, Kim, Verfaillie, Catherine, De Muynck, Louis, Guo, Wenting, Vandenberghe, Rik, Van Den Bosch, Ludo, Van Damme, Philip, Espuny Camacho, Ira Mercedes, Vanderhaeghen, Pierre, Tóth, Balázs, Voets, Thomas, and Cathomen, Toni

Abstract

To understand how haploinsufficiency of progranulin (PGRN) causes frontotemporal dementia (FTD), we created induced pluripotent stem cells (iPSCs) from patients carrying the GRNIVS1+5G > C mutation (FTD-iPSCs). FTD-iPSCs were fated to cortical neurons, the cells most affected in FTD. Although generation of neuroprogenitors was unaffected, their further differentiation into CTIP2-, FOXP2-, or TBR1-TUJ1 double-positive cortical neurons, but not motorneurons, was significantly decreased in FTD-neural progeny. Zinc finger nuclease-mediated introduction of GRN cDNA into the AAVS1 locus corrected defects in cortical neurogenesis, demonstrating that PGRN haploinsufficiency causes inefficient cortical neuron generation. RNA sequencing analysis confirmed reversal of the altered gene expression profile following genetic correction. We identified the Wnt signaling pathway as one of the top defective pathways in FTD-iPSC-derived neurons, which was reversed following genetic correction. Differentiation of FTD-iPSCs in the presence of a WNT inhibitor mitigated defective corticogenesis. Therefore, we demonstrate that PGRN haploinsufficiency hampers corticogenesis in vitro., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
2015

10. Restoration of Progranulin Expression Rescues Cortical Neuron Generation in an Induced Pluripotent Stem Cell Model of Frontotemporal Dementia

Author

Raitano, Susanna, primary, Ordovàs, Laura, additional, De Muynck, Louis, additional, Guo, Wenting, additional, Espuny-Camacho, Ira, additional, Geraerts, Martine, additional, Khurana, Satish, additional, Vanuytsel, Kim, additional, Tóth, Balazs I., additional, Voets, Thomas, additional, Vandenberghe, Rik, additional, Cathomen, Toni, additional, Van Den Bosch, Ludo, additional, Vanderhaeghen, Pierre, additional, Van Damme, Philip, additional, and Verfaillie, Catherine M., additional

Published
2015
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11. AN ACADEMIC-PRIVATE PARTNERSHIP FOR THE VALIDATION OF NEW MODELS TO UNDERSTAND TAU-RELATED HYPEREXCITABILITY AND AGGREGATION USING HUMAN-INDUCED PLURIPOTENT STEM CELLS

Author

Garcia Leon, Juan Antonio, Pestana, Francisco, Kreir, Mohamed, Shih, Pei-Yu, Eggermont, Kristel, Terryn, Joke, Stumman, Tina Charlotte, Geraerts, Martine, Craig, Peter James, Nuthall, Hugh norman, Verheyen, An, Royaux, Ines, Fazal, Raheem, Quiles, Ana, Ordovas, Laura, Bruce, Kevin, Foerch, Patrik, Pita Almenar, Juan Diego, Ebneth, Andreas, Verfaillie, Catherine, and Socorro, Alfredo Cabrera

Published
2017
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