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Bénédicte Neven
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Journal Articles

Mutations disrupting the kinase domain of IKKα lead to immunodeficiency and immune dysregulation in humans

Quentin Riller, Boris Sorin, Charline Courteille, Duong Ho-Nhat, Tom Le Voyer, Jean-Christophe Debray, Marie-Claude Stolzenberg, Muriel Schmutz, Olivier Pellé, Thomas Becquard, María Rodrigo Riestra, Laureline Berteloot, Mélanie Migaud, Laure Delage, Marie Jeanpierre, Charlotte Boussard, Camille Brunaud, Aude Magérus, Charles Bretot, Victor Michel, Camille Roux, Capucine Picard, Cécile Masson, Christine Bole-Feysot, Nicolas Cagnard, Aurélien Corneau, Isabelle Meyts, Véronique Baud, Jean-Laurent Casanova, Alain Fischer, Emmanuel Dejardin, Anne Puel, Cécile Boulanger, Bénédicte Neven, Frédéric Rieux-Laucat
Journal: Journal of Experimental Medicine
J Exp Med (2025) 222 (2): e20240843.
https://doi.org/10.1084/jem.20240843
Published: 15 January 2025
View article titled, Mutations disrupting the kinase domain of IKKα lead to immunodeficiency and immune dysregulation in humans
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Includes: Supplementary data
Journal Articles

Helper T cell immunity in humans with inherited CD4 deficiency

In Special Collection:
Journal-Based CME Activities with Journal of Experimental Medicine (JEM) , Thymus, T Cell Development, and Inborn Errors
Antoine Guérin, Marcela Moncada-Vélez, Katherine Jackson, Masato Ogishi, Jérémie Rosain, Mathieu Mancini, David Langlais, Andrea Nunez, Samantha Webster, Jesse Goyette, Taushif Khan, Nico Marr, Danielle T. Avery, Geetha Rao, Tim Waterboer, Birgitta Michels, Esmeralda Neves, Cátia Iracema Morais, Jonathan London, Stéphanie Mestrallet, Pierre Quartier dit Maire, Bénédicte Neven, Franck Rapaport, Yoann Seeleuthner, Atar Lev, Amos J. Simon, Jorge Montoya, Ortal Barel, Julio Gómez-Rodríguez, Julio C. Orrego, Anne-Sophie L’Honneur, Camille Soudée, Jessica Rojas, Alejandra C. Velez, Irini Sereti, Benjamin Terrier, Nancy Marin, Luis F. García, Laurent Abel, Stéphanie Boisson-Dupuis, Joel Reis, Antonio Marinho, Andrea Lisco, Emilia Faria, Christopher C. Goodnow, Julia Vasconcelos, Vivien Béziat, Cindy S. Ma, Raz Somech, Jean-Laurent Casanova, Jacinta Bustamante, Jose Luis Franco, Stuart G. Tangye
Journal: Journal of Experimental Medicine
J Exp Med (2024) 221 (5): e20231044.
https://doi.org/10.1084/jem.20231044
Published: 01 April 2024
View article titled, Helper T cell immunity in humans with inherited CD4 deficiency
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Includes: Supplementary data
Journal Articles

Higher COVID-19 pneumonia risk associated with anti-IFN-α than with anti-IFN-ω auto-Abs in children

In Special Collection:
Innate Immune Signaling: From Host Defense to Disease Pathogenesis
Paul Bastard, Adrian Gervais, Maki Taniguchi, Liisa Saare, Karita Särekannu, Tom Le Voyer, Quentin Philippot, Jérémie Rosain, Lucy Bizien, Takaki Asano, Marina Garcia-Prat, Alba Parra-Martínez, Mélanie Migaud, Miyuki Tsumura, Francesca Conti, Alexandre Belot, Jacques G. Rivière, Tomohiro Morio, Junko Tanaka, Etienne Javouhey, Filomeen Haerynck, Sotirija Duvlis, Tayfun Ozcelik, Sevgi Keles, Yacine Tandjaoui-Lambiotte, Simon Escoda, Maya Husain, Qiang Pan-Hammarström, Lennart Hammarström, Gloria Ahlijah, Anthony Abi Haidar, Camille Soudee, Vincent Arseguel, Hassan Abolhassani, Sabina Sahanic, Ivan Tancevski, Yoko Nukui, Seiichi Hayakawa, George P. Chrousos, Athanasios Michos, Elizabeth-Barbara Tatsi, Filippos Filippatos, Agusti Rodriguez-Palmero, Jesus Troya, Imran Tipu, Isabelle Meyts, Lucie Roussel, Sisse Rye Ostrowski, Laire Schidlowski, Carolina Prando, Antonio Condino-Neto, Nathalie Cheikh, Ahmed A. Bousfiha, Jalila El Bakkouri, COVID Clinicians, GEN-COVID Study Group, COVID Human Genetic Effort, Pärt Peterson, Aurora Pujol, Romain Lévy, Pierre Quartier, Donald C. Vinh, Bertrand Boisson, Vivien Béziat, Shen-Ying Zhang, Alessandro Borghesi, Andrea Pession, Evangelos Andreakos, Nico Marr, Alexios-Fotios A. Mentis, Trine H. Mogensen, Carlos Rodríguez-Gallego, Pere Soler-Palacin, Roger Colobran, Vallo Tillmann, Bénédicte Neven, Sophie Trouillet-Assant, Petter Brodin, Laurent Abel, Emmanuelle Jouanguy, Qian Zhang, Federico Martinón-Torres, Antonio Salas, Alberto Gómez-Carballa, Luis I. Gonzalez-Granado, Kai Kisand, Satoshi Okada, Anne Puel, Aurélie Cobat, Jean-Laurent Casanova
Journal: Journal of Experimental Medicine
J Exp Med (2024) 221 (2): e20231353.
https://doi.org/10.1084/jem.20231353
Published: 04 January 2024
View article titled, Higher COVID-19 pneumonia risk associated with anti-IFN-α than with anti-IFN-ω auto-Abs in children
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Journal Articles

Human CARMIL2 deficiency underlies a broader immunological and clinical phenotype than CD28 deficiency

Romain Lévy, Florian Gothe, Mana Momenilandi, Thomas Magg, Marie Materna, Philipp Peters, Johannes Raedler, Quentin Philippot, Anita Lena Rack-Hoch, David Langlais, Mathieu Bourgey, Anna-Lisa Lanz, Masato Ogishi, Jérémie Rosain, Emmanuel Martin, Sylvain Latour, Natasha Vladikine, Marco Distefano, Taushif Khan, Franck Rapaport, Marian S. Schulz, Ursula Holzer, Anders Fasth, Georgios Sogkas, Carsten Speckmann, Arianna Troilo, Venetia Bigley, Anna Roppelt, Yael Dinur-Schejter, Ori Toker, Karen Helene Bronken Martinsen, Roya Sherkat, Ido Somekh, Raz Somech, Dror S. Shouval, Jörn-Sven Kühl, Winnie Ip, Elizabeth M. McDermott, Lucy Cliffe, Ahmet Ozen, Safa Baris, Hemalatha G. Rangarajan, Emmanuelle Jouanguy, Anne Puel, Jacinta Bustamante, Marie-Alexandra Alyanakian, Mathieu Fusaro, Yi Wang, Xiao-Fei Kong, Aurélie Cobat, David Boutboul, Martin Castelle, Claire Aguilar, Olivier Hermine, Morgane Cheminant, Felipe Suarez, Alisan Yildiran, Aziz Bousfiha, Hamoud Al-Mousa, Fahad Alsohime, Deniz Cagdas, Roshini S. Abraham, Alan P. Knutsen, Borre Fevang, Sagar Bhattad, Ayca Kiykim, Baran Erman, Tugba Arikoglu, Ekrem Unal, Ashish Kumar, Christoph B. Geier, Ulrich Baumann, Bénédicte Neven, CARMIL2 Consortium, Meino Rohlfs, Christoph Walz, Laurent Abel, Bernard Malissen, Nico Marr, Christoph Klein, Jean-Laurent Casanova, Fabian Hauck, Vivien Béziat
Journal: Journal of Experimental Medicine
J Exp Med (2022) 220 (2): e20220275.
https://doi.org/10.1084/jem.20220275
Published: 14 December 2022
View article titled, Human CARMIL2 deficiency underlies a broader immunological and clinical phenotype than CD28 deficiency
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Includes: Supplementary data
Journal Articles

Human type I IFN deficiency does not impair B cell response to SARS-CoV-2 mRNA vaccination

In Special Collection:
B Cell Biology in Health and Diseases
Aurélien Sokal, Paul Bastard, Pascal Chappert, Giovanna Barba-Spaeth, Slim Fourati, Alexis Vanderberghe, Pauline Lagouge-Roussey, Isabelle Meyts, Adrian Gervais, Magali Bouvier-Alias, Imane Azzaoui, Ignacio Fernández, Andréa de la Selle, Qian Zhang, Lucy Bizien, Isabelle Pellier, Agnès Linglart, Anya Rothenbuhler, Estelle Marcoux, Raphael Anxionnat, Nathalie Cheikh, Juliane Léger, Blanca Amador-Borrero, Fanny Fouyssac, Vanessa Menut, Jean-Christophe Goffard, Caroline Storey, Caroline Demily, Coralie Mallebranche, Jesus Troya, Aurora Pujol, Marie Zins, Pierre Tiberghien, Paul E. Gray, Peter McNaughton, Anna Sullivan, Jane Peake, Romain Levy, Laetitia Languille, Carlos Rodiguez-Gallego, Bertrand Boisson, Sébastien Gallien, Bénédicte Neven, Marc Michel, Bertrand Godeau, Laurent Abel, Felix A. Rey, Jean-Claude Weill, Claude-Agnès Reynaud, Stuart G. Tangye, Jean-Laurent Casanova, Matthieu Mahévas
Journal: Journal of Experimental Medicine
J Exp Med (2022) 220 (1): e20220258.
https://doi.org/10.1084/jem.20220258
Published: 07 November 2022
View article titled, Human type I IFN deficiency does not impair B cell response to SARS-CoV-2 mRNA vaccination
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Includes: Supplementary data
Journal Articles

Inherited human ITK deficiency impairs IFN-γ immunity and underlies tuberculosis

In Special Collection:
Thymus, T Cell Development, and Inborn Errors
Masato Ogishi, Rui Yang, Rémy Rodriguez, Dominic P. Golec, Emmanuel Martin, Quentin Philippot, Jonathan Bohlen, Simon J. Pelham, Andrés Augusto Arias, Taushif Khan, Manar Ata, Fatima Al Ali, Flore Rozenberg, Xiao-Fei Kong, Maya Chrabieh, Candice Laine, Wei-Te Lei, Ji Eun Han, Yoann Seeleuthner, Zenia Kaul, Emmanuelle Jouanguy, Vivien Béziat, Leila Youssefian, Hassan Vahidnezhad, V. Koneti Rao, Bénédicte Neven, Claire Fieschi, Davood Mansouri, Mohammad Shahrooei, Sevgi Pekcan, Gulsum Alkan, Melike Emiroğlu, Hüseyin Tokgöz, Jouni Uitto, Fabian Hauck, Jacinta Bustamante, Laurent Abel, Sevgi Keles, Nima Parvaneh, Nico Marr, Pamela L. Schwartzberg, Sylvain Latour, Jean-Laurent Casanova, Stéphanie Boisson-Dupuis
Journal: Journal of Experimental Medicine
J Exp Med (2022) 220 (1): e20220484.
https://doi.org/10.1084/jem.20220484
Published: 03 November 2022
View article titled, Inherited human ITK deficiency impairs IFN-γ immunity and underlies tuberculosis
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Includes: Supplementary data
Journal Articles

Inherited TNFSF9 deficiency causes broad Epstein–Barr virus infection with EBV+ smooth muscle tumors

In Special Collection:
Mechanisms and Models of Cancer 2022 Update
Benjamin Fournier, Akihiro Hoshino, Julie Bruneau, Camille Bachelet, Mathieu Fusaro, Roman Klifa, Romain Lévy, Christelle Lenoir, Claire Soudais, Capucine Picard, Stéphane Blanche, Martin Castelle, Despina Moshous, Thierry Molina, Anne-Sophie Defachelles, Bénédicte Neven, Sylvain Latour
Journal: Journal of Experimental Medicine
J Exp Med (2022) 219 (7): e20211682.
https://doi.org/10.1084/jem.20211682
Published: 03 June 2022
View article titled, Inherited TNFSF9 deficiency causes broad Epstein–Barr virus infection with EBV<sup>+</sup> smooth muscle tumors
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Includes: Supplementary data
Journal Articles

Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1

In Special Collection:
Cytokines Collection 2021 , Innate Immunity: From Basic Biology to Human Disease , JEM Clinical Immunology Collection 2021
Paul Bastard, Elizaveta Orlova, Leila Sozaeva, Romain Lévy, Alyssa James, Monica M. Schmitt, Sebastian Ochoa, Maria Kareva, Yulia Rodina, Adrian Gervais, Tom Le Voyer, Jérémie Rosain, Quentin Philippot, Anna-Lena Neehus, Elana Shaw, Mélanie Migaud, Lucy Bizien, Olov Ekwall, Stefan Berg, Guglielmo Beccuti, Lucia Ghizzoni, Gérard Thiriez, Arthur Pavot, Cécile Goujard, Marie-Louise Frémond, Edwin Carter, Anya Rothenbuhler, Agnès Linglart, Brigite Mignot, Aurélie Comte, Nathalie Cheikh, Olivier Hermine, Lars Breivik, Eystein S. Husebye, Sébastien Humbert, Pierre Rohrlich, Alain Coaquette, Fanny Vuoto, Karine Faure, Nizar Mahlaoui, Primož Kotnik, Tadej Battelino, Katarina Trebušak Podkrajšek, Kai Kisand, Elise M.N. Ferré, Thomas DiMaggio, Lindsey B. Rosen, Peter D. Burbelo, Martin McIntyre, Nelli Y. Kann, Anna Shcherbina, Maria Pavlova, Anna Kolodkina, Steven M. Holland, Shen-Ying Zhang, Yanick J. Crow, Luigi D. Notarangelo, Helen C. Su, Laurent Abel, Mark S. Anderson, Emmanuelle Jouanguy, Bénédicte Neven, Anne Puel, Jean-Laurent Casanova, Michail S. Lionakis
Journal: Journal of Experimental Medicine
J Exp Med (2021) 218 (7): e20210554.
https://doi.org/10.1084/jem.20210554
Published: 23 April 2021
View article titled, Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1
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Includes: Supplementary data
Journal Articles

Rapid identification and characterization of infected cells in blood during chronic active Epstein-Barr virus infection

In Special Collection:
2020 Clinical Collection on Viral Infections
Benjamin Fournier, David Boutboul, Julie Bruneau, Charline Miot, Cécile Boulanger, Marion Malphettes, Isabelle Pellier, Bertrand Dunogué, Benjamin Terrier, Felipe Suarez, Stéphane Blanche, Martin Castelle, Sarah Winter, Henri-Jacques Delecluse, Thierry Molina, Capucine Picard, Stephan Ehl, Despina Moshous, Lionel Galicier, Vincent Barlogis, Alain Fischer, Bénédicte Neven, Sylvain Latour
Journal: Journal of Experimental Medicine
J Exp Med (2020) 217 (11): e20192262.
https://doi.org/10.1084/jem.20192262
Published: 18 August 2020
View article titled, Rapid identification and characterization of infected cells in blood during chronic active Epstein-Barr virus infection
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Includes: Supplementary data
Journal Articles

Mutations in COPA lead to abnormal trafficking of STING to the Golgi and interferon signaling

In Special Collection:
Innate Immune Signaling in Disease and Homeostasis
Alice Lepelley, Maria José Martin-Niclós, Melvin Le Bihan, Joseph A. Marsh, Carolina Uggenti, Gillian I. Rice, Vincent Bondet, Darragh Duffy, Jonny Hertzog, Jan Rehwinkel, Serge Amselem, Siham Boulisfane-El Khalifi, Mary Brennan, Edwin Carter, Lucienne Chatenoud, Stéphanie Chhun, Aurore Coulomb l’Hermine, Marine Depp, Marie Legendre, Karen J. Mackenzie, Jonathan Marey, Catherine McDougall, Kathryn J. McKenzie, Thierry Jo Molina, Bénédicte Neven, Luis Seabra, Caroline Thumerelle, Marie Wislez, Nadia Nathan, Nicolas Manel, Yanick J. Crow, Marie-Louise Frémond
Journal: Journal of Experimental Medicine
J Exp Med (2020) 217 (11): e20200600.
https://doi.org/10.1084/jem.20200600
Published: 28 July 2020
View article titled, Mutations in <em>COPA</em> lead to abnormal trafficking of STING to the Golgi and interferon signaling
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Includes: Supplementary data
Journal Articles

Concomitant PIK3CD and TNFRSF9 deficiencies cause chronic active Epstein-Barr virus infection of T cells

Rémy Rodriguez, Benjamin Fournier, Debora Jorge Cordeiro, Sarah Winter, Kazushi Izawa, Emmanuel Martin, David Boutboul, Christelle Lenoir, Sylvie Fraitag, Sven Kracker, Tania H. Watts, Capucine Picard, Julie Bruneau, Isabelle Callebaut, Alain Fischer, Bénédicte Neven, Sylvain Latour
Journal: Journal of Experimental Medicine
J Exp Med (2019) 216 (12): 2800–2818.
https://doi.org/10.1084/jem.20190678
Published: 19 September 2019
View article titled, Concomitant <em>PIK3CD</em> and <em>TNFRSF9</em> deficiencies cause chronic active Epstein-Barr virus infection of T cells
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Includes: Supplementary data
Journal Articles

Protein kinase D at the Golgi controls NLRP3 inflammasome activation

Zhirong Zhang, Gergö Meszaros, Wan-ting He, Yanfang Xu, Helena de Fatima Magliarelli, Laurent Mailly, Michael Mihlan, Yansheng Liu, Marta Puig Gámez, Alexander Goginashvili, Adrien Pasquier, Olga Bielska, Bénédicte Neven, Pierre Quartier, Rudolf Aebersold, Thomas F. Baumert, Philippe Georgel, Jiahuai Han, Romeo Ricci
Journal: Journal of Experimental Medicine
J Exp Med (2017) 214 (9): 2671–2693.
https://doi.org/10.1084/jem.20162040
Published: 17 July 2017
View article titled, Protein kinase D at the Golgi controls NLRP3 inflammasome activation
Includes: Supplementary data
Journal Articles

Intrinsic antiproliferative activity of the innate sensor STING in T lymphocytes

In Special Collection:
Monogenic Auto-Inflammation and Auto-Immunity in Humans 2018
Silvia Cerboni, Nadia Jeremiah, Matteo Gentili, Ulf Gehrmann, Cécile Conrad, Marie-Claude Stolzenberg, Capucine Picard, Bénédicte Neven, Alain Fischer, Sébastian Amigorena, Frédéric Rieux-Laucat, Nicolas Manel
Journal: Journal of Experimental Medicine
J Exp Med (2017) 214 (6): 1769–1785.
https://doi.org/10.1084/jem.20161674
Published: 08 May 2017
View article titled, Intrinsic antiproliferative activity of the innate sensor STING in T lymphocytes
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Includes: Supplementary data
Journal Articles

Detection of interferon alpha protein reveals differential levels and cellular sources in disease

In Special Collection:
Monogenic Auto-Inflammation and Auto-Immunity in Humans 2018
Mathieu P. Rodero, Jérémie Decalf, Vincent Bondet, David Hunt, Gillian I. Rice, Scott Werneke, Sarah L. McGlasson, Marie-Alexandra Alyanakian, Brigitte Bader-Meunier, Christine Barnerias, Nathalia Bellon, Alexandre Belot, Christine Bodemer, Tracy A. Briggs, Isabelle Desguerre, Marie-Louise Frémond, Marie Hully, Arn M.J.M. van den Maagdenberg, Isabelle Melki, Isabelle Meyts, Lucile Musset, Nadine Pelzer, Pierre Quartier, Gisela M. Terwindt, Joanna Wardlaw, Stewart Wiseman, Frédéric Rieux-Laucat, Yoann Rose, Bénédicte Neven, Christina Hertel, Adrian Hayday, Matthew L. Albert, Flore Rozenberg, Yanick J. Crow, Darragh Duffy
Journal: Journal of Experimental Medicine
J Exp Med (2017) 214 (5): 1547–1555.
https://doi.org/10.1084/jem.20161451
Published: 18 April 2017
View article titled, Detection of interferon alpha protein reveals differential levels and cellular sources in disease
Open the PDF for Detection of interferon alpha protein reveals differential levels and cellular sources in disease in another window
Includes: Supplementary data