Are Frontal Cognitive and Atrophy Patterns Different in PSP and bvFTD? A Comparative Neuropsychological and VBM Study

por Lagarde, Julien, Valabrègue, Romain, Corvol, Jean-Christophe, Pineau, Fanny, Le Ber, Isabelle, Vidailhet, Marie, Dubois, Bruno, Levy, Richard
Publicado 2013

Cognitive and behavioural inhibition deficits in neurodegenerative dementias

por Migliaccio, Raffaella, Tanguy, Delphine, Bouzigues, Arabella, Sezer, Idil, Dubois, Bruno, Le Ber, Isabelle, Batrancourt, Bénédicte, Godefroy, Valérie, Levy, Richard
Publicado 2020

MicroRNA signatures in genetic frontotemporal dementia and amyotrophic lateral sclerosis

por Kmetzsch, Virgilio, Latouche, Morwena, Saracino, Dario, Rinaldi, Daisy, Camuzat, Agnès, Gareau, Thomas, Le Ber, Isabelle, Colliot, Olivier, Becker, Emmanuelle
Publicado 2022

White matter lesions in FTLD: distinct phenotypes characterize GRN and C9ORF72 mutations

por Ameur, Fatima, Colliot, Olivier, Caroppo, Paola, Ströer, Sebastian, Dormont, Didier, Brice, Alexis, Azuar, Carole, Dubois, Bruno, Le Ber, Isabelle, Bertrand, Anne
Publicado 2016

New Antibody-Free Mass Spectrometry-Based Quantification Reveals That C9ORF72 Long Protein Isoform Is Reduced in the Frontal Cortex of Hexanucleotide-Repeat Expansion Carriers

por Viodé, Arthur, Fournier, Clémence, Camuzat, Agnès, Fenaille, François, Latouche, Morwena, Elahi, Fanny, Le Ber, Isabelle, Junot, Christophe, Lamari, Foudil, Anquetil, Vincent, Becher, François
Publicado 2018

Who and Why? Requests for Presymptomatic Genetic Testing for Amyotrophic Lateral Sclerosis/Frontotemporal Dementia vs Huntington Disease

por Amador, Maria del Mar, Gargiulo, Marcela, Boucher, Christilla, Herson, Ariane, Staraci, Stéphanie, Salachas, François, Clot, Fabienne, Cazeneuve, Cécile, Le Ber, Isabelle, Durr, Alexandra
Publicado 2020

Pathogenic p62/SQSTM1 mutations impair energy metabolism through limitation of mitochondrial substrates

por Bartolome, Fernando, Esteras, Noemi, Martin-Requero, Angeles, Boutoleau-Bretonniere, Claire, Vercelletto, Martine, Gabelle, Audrey, Le Ber, Isabelle, Honda, Tadashi, Dinkova-Kostova, Albena T., Hardy, John, Carro, Eva, Abramov, Andrey Y.
Publicado 2017

de novo MAPT mutation G335A causes severe brain atrophy, 3R and 4R PHF-tau pathology and early onset frontotemporal dementia

por Ando, Kunie, Ferlini, Lorenzo, Suain, Valérie, Yilmaz, Zehra, Mansour, Salwa, Le Ber, Isabelle, Bouchard, Cécile, Leroy, Karelle, Durr, Alexandra, Clot, Fabienne, Sarazin, Marie, Bier, Jean-Christophe, Brion, Jean-Pierre
Publicado 2020

Factors influencing the age at onset in familial frontotemporal lobar dementia: Important weight of genetics

por Barbier, Mathieu, Camuzat, Agnès, Houot, Marion, Clot, Fabienne, Caroppo, Paola, Fournier, Clémence, Rinaldi, Daisy, Pasquier, Florence, Hannequin, Didier, Pariente, Jérémie, Larcher, Kathy, Brice, Alexis, Génin, Emmanuelle, Sabbagh, Audrey, Le Ber, Isabelle
Publicado 2017

Interrupted CAG expansions in ATXN2 gene expand the genetic spectrum of frontotemporal dementias

por Fournier, Clémence, Anquetil, Vincent, Camuzat, Agnès, Stirati-Buron, Sandrine, Sazdovitch, Véronique, Molina-Porcel, Laura, Turbant, Sabrina, Rinaldi, Daisy, Sánchez-Valle, Raquel, Barbier, Mathieu, Latouche, Morwena, Stevanin, Giovanni, Seilhean, Danielle, Brice, Alexis, Duyckaerts, Charles, Le Ber, Isabelle
Publicado 2018

Characteristics and progression of patients with frontotemporal dementia in a regional memory clinic network

por Leroy, Mélanie, Bertoux, Maxime, Skrobala, Emilie, Mode, Elisa, Adnet-Bonte, Catherine, Le Ber, Isabelle, Bombois, Stéphanie, Cassagnaud, Pascaline, Chen, Yaohua, Deramecourt, Vincent, Lebert, Florence, Mackowiak, Marie Anne, Sillaire, Adeline Rollin, Wathelet, Marielle, Pasquier, Florence, Lebouvier, Thibaud
Publicado 2021

Semantic and nonfluent aphasic variants, secondarily associated with amyotrophic lateral sclerosis, are predominant frontotemporal lobar degeneration phenotypes in TBK1 carriers

por Caroppo, Paola, Camuzat, Agnès, De Septenville, Anne, Couratier, Philippe, Lacomblez, Lucette, Auriacombe, Sophie, Flabeau, Olivier, Jornéa, Ludmila, Blanc, Frederic, Sellal, François, Cretin, Benjamin, Meininger, Vincent, Fleury, Marie-Céline, Couarch, Philippe, Dubois, Bruno, Brice, Alexis, Le Ber, Isabelle
Publicado 2015

Reduced Tau protein expression is associated with frontotemporal degeneration with progranulin mutation

por Papegaey, Anthony, Eddarkaoui, Sabiha, Deramecourt, Vincent, Fernandez-Gomez, Francisco-Jose, Pantano, Pierre, Obriot, Hélène, Machala, Camille, Anquetil, Vincent, Camuzat, Agnès, Brice, Alexis, Maurage, Claude-Alain, Le Ber, Isabelle, Duyckaerts, Charles, Buée, Luc, Sergeant, Nicolas, Buée-Scherrer, Valérie
Publicado 2016

Plasma microRNA signature in presymptomatic and symptomatic subjects with C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis

por Kmetzsch, Virgilio, Anquetil, Vincent, Saracino, Dario, Rinaldi, Daisy, Camuzat, Agnès, Gareau, Thomas, Jornea, Ludmila, Forlani, Sylvie, Couratier, Philippe, Wallon, David, Pasquier, Florence, Robil, Noémie, de la Grange, Pierre, Moszer, Ivan, Le Ber, Isabelle, Colliot, Olivier, Becker, Emmanuelle
Publicado 2021

An ecological approach to identify distinct neural correlates of disinhibition in frontotemporal dementia

por Tanguy, Delphine, Batrancourt, Bénédicte, Estudillo-Romero, Alfonso, Baxter, John S.H., Le Ber, Isabelle, Bouzigues, Arabella, Godefroy, Valérie, Funkiewiez, Aurélie, Chamayou, Céline, Volle, Emmanuelle, Saracino, Dario, Rametti-Lacroux, Armelle, Morandi, Xavier, Jannin, Pierre, Levy, Richard, Migliaccio, Raffaella
Publicado 2022

Defining the spectrum of frontotemporal dementias associated with TARDBP mutations

por Caroppo, Paola, Camuzat, Agnès, Guillot-Noel, Léna, Thomas-Antérion, Catherine, Couratier, Philippe, Wong, Tsz Hang, Teichmann, Marc, Golfier, Véronique, Auriacombe, Sophie, Belliard, Serge, Laurent, Bernard, Lattante, Serena, Millecamps, Stéphanie, Clot, Fabienne, Dubois, Bruno, van Swieten, John C., Brice, Alexis, Le Ber, Isabelle
Publicado 2016

Genome-wide expression profiling and functional characterization of SCA28 lymphoblastoid cell lines reveal impairment in cell growth and activation of apoptotic pathways

por Mancini, Cecilia, Roncaglia, Paola, Brussino, Alessandro, Stevanin, Giovanni, Lo Buono, Nicola, Krmac, Helena, Maltecca, Francesca, Gazzano, Elena, Bartoletti Stella, Anna, Calvaruso, Maria Antonietta, Iommarini, Luisa, Cagnoli, Claudia, Forlani, Sylvie, Le Ber, Isabelle, Durr, Alexandra, Brice, Alexis, Ghigo, Dario, Casari, Giorgio, Porcelli, Anna Maria, Funaro, Ada, Gasparre, Giuseppe, Gustincich, Stefano, Brusco, Alfredo
Publicado 2013

C9ORF72 knockdown triggers FTD-like symptoms and cell pathology in mice

por Lopez-Herdoiza, Maria-Belen, Bauché, Stephanie, Wilmet, Baptiste, Le Duigou, Caroline, Roussel, Delphine, Frah, Magali, Béal, Jonas, Devely, Gabin, Boluda, Susana, Frick, Petra, Bouteiller, Delphine, Dussaud, Sébastien, Guillabert, Pierre, Dalle, Carine, Dumont, Magali, Camuzat, Agnes, Saracino, Dario, Barbier, Mathieu, Bruneteau, Gaelle, Ravassard, Phillippe, Neumann, Manuela, Nicole, Sophie, Le Ber, Isabelle, Brice, Alexis, Latouche, Morwena
Publicado 2023

Lateral Temporal Lobe: An Early Imaging Marker of the Presymptomatic GRN Disease?

por Caroppo, Paola, Habert, Marie-Odile, Durrleman, Stanley, Funkiewiez, Aurélie, Perlbarg, Vincent, Hahn, Valérie, Bertin, Hugo, Gaubert, Malo, Routier, Alexandre, Hannequin, Didier, Deramecourt, Vincent, Pasquier, Florence, Rivaud-Pechoux, Sophie, Vercelletto, Martine, Edouart, Geoffrey, Valabregue, Romain, Lejeune, Pascal, Didic, Mira, Corvol, Jean-Christophe, Benali, Habib, Lehericy, Stephane, Dubois, Bruno, Colliot, Olivier, Brice, Alexis, Le Ber, Isabelle
Publicado 2015

Plasma NfL levels and longitudinal change rates in C9orf72 and GRN-associated diseases: from tailored references to clinical applications

por Saracino, Dario, Dorgham, Karim, Camuzat, Agnès, Rinaldi, Daisy, Rametti-Lacroux, Armelle, Houot, Marion, Clot, Fabienne, Martin-Hardy, Philippe, Jornea, Ludmila, Azuar, Carole, Migliaccio, Raffaella, Pasquier, Florence, Couratier, Philippe, Auriacombe, Sophie, Sauvée, Mathilde, Boutoleau-Bretonnière, Claire, Pariente, Jérémie, Didic, Mira, Hannequin, Didier, Wallon, David, Colliot, Olivier, Dubois, Bruno, Brice, Alexis, Levy, Richard, Forlani, Sylvie, Le Ber, Isabelle
Publicado 2021