Paul Bastard

Jul 24, 2024 | nature.com | Yi-Hao Chan |zhiyong Liu |Paul Bastard |Oliver Harschnitz |Peng Zhang |Gabriele Ciceri | +14 more

AbstractMost cases of herpes simplex virus 1 (HSV-1) encephalitis (HSE) remain unexplained1,2. Here, we report on two unrelated people who had HSE as children and are homozygous for rare deleterious variants of TMEFF1, which encodes a cell membrane protein that is preferentially expressed by brain cortical neurons. TMEFF1 interacts with the cell-surface HSV-1 receptor NECTIN-1, impairing HSV-1 glycoprotein D- and NECTIN-1-mediated fusion of the virus and the cell membrane, blocking viral entry.

Apr 28, 2024 | onlinelibrary.wiley.com | Paul Bastard |Nathalie Cozic |Régis Brion |Nathalie Gaspar

CONFLICT OF INTEREST STATEMENT The authors have no conflicts of interest to declare in regards to the contents of the article. Supporting Information Filename Description pbc31029-sup-0001-TableS1-S3.docx31.7 KB Supporting Information pbc31029-sup-0002-TableS4.docx24.7 KB Supporting Information pbc31029-sup-0003-FigureS1.pdf187.3 KB Figure S1 Evolution of lymphocyte count during the course of the treatment (in the first 46 patients studied).

Feb 27, 2024 | zenodo.org | Al Ali |Le Voyer |Paul Bastard |Thangavel Alphonse |Al Qureshah |Seyed Mohammad | +4 more

We describe humans with rare biallelic loss-of-function PTCRA variants impairing pre–a T cell receptor (pre-TCRa) expression. Low circulating naive ab T cell counts at birth persisted over time, with normal memory ab and high gd T cell counts. Their TCRa repertoire was biased, which suggests that noncanonical thymic differentiation pathways can rescue ab T cell development. Only a minority of these individuals were sick, with infection, lymphoproliferation, and/or autoimmunity.

Jan 9, 2024 | onlinelibrary.wiley.com | Paul Bastard |Paris Sorbonne Cité |Giles Laboratory |Pediatric Hematology-Immunology

1 INTRODUCTION Not all individuals exposed to any given pathogen become infected and the impact of infection is highly variable, from silent infection to lethal disease. Many historically lethal infections can now be prevented or cured with modern medicine.

Dec 22, 2023 | science.org | Paul Bastard |Nora Ruef |Li Ran |Jose Martínez Magdaleno

Research Article CELL MIGRATION Nora Ruef https://orcid.org/0000-0001-5211-3363, Jose Martínez Magdaleno https://orcid.org/0000-0001-6366-5892, [...] , Xenia Ficht https://orcid.org/0000-0002-4534-8225, Vladimir Purvanov, [...] , Matthieu Palayret https://orcid.org/0000-0002-5343-8378, Stefanie Wissmann, Petra Pfenninger https://orcid.org/0000-0003-1152-5278, Bettina Stolp https://orcid.org/0000-0003-2945-8536, Flavian Thelen, [...] , Juliana Barreto de Albuquerque...