{"entity": "researcher", "timestamp": "2026-08-20T21:15:17.659Z", "family": "Bastard", "given": "Paul", "initials": "P", "orcid": "0000-0002-5926-8437", "affiliations": ["Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM) U1163, Necker Hospital for Sick Children , Paris, France.", "Paris Cit\u00e9 University, Imagine Institute , Paris, France.", "St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University , New York, NY, USA.", "Pediatric Hematology-Immunology and Rheumatology Unit, Necker Hospital for Sick Children, AP-HP , Paris, France."], "links": {"self": {"href": "https://publications-affiliated.scilifelab.se/researcher/beec83103d55467291f2b8c160df3a04.json"}, "display": {"href": "https://publications-affiliated.scilifelab.se/researcher/beec83103d55467291f2b8c160df3a04"}}, "publications": [{"entity": "publication", "iuid": 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"given": "Francesca", "initials": "F", "orcid": "0009-0007-7206-6796", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/d3f2dc1b82df4c5eb55cdb622514e56e.json"}}, {"family": "Codullo", "given": "Veronica", "initials": "V", "orcid": "0000-0003-2557-8514", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/5fd864a526fa48faa6b9b4f65cbf7a06.json"}}, {"family": "Cavagna", "given": "Lorenzo", "initials": "L", "orcid": "0000-0003-3292-1528", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/fb86f8c9a78b40d9a653b4c6d3727c4d.json"}}, {"family": "Schleinitz", "given": "Nicolas", "initials": "N", "orcid": "0000-0001-9449-3392", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/549ab52a38094bee9bec4d184d309695.json"}}, {"family": "Bousfiha", "given": "Mohamed", "initials": "M", "orcid": "0009-0002-3099-1127", "researcher": {"href": 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"Trine H", "initials": "TH", "orcid": "0000-0002-1853-9704", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/a419fc2fa6c54620900fb1c9c674cc5d.json"}}, {"family": "Bastard", "given": "Paul", "initials": "P", "orcid": "0000-0002-5926-8437", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/beec83103d55467291f2b8c160df3a04.json"}}, {"family": "Puel", "given": "Anne", "initials": "A", "orcid": "0000-0003-2603-0323", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/d106604b8ad84e67870302b906a94963.json"}}, {"family": "Borghesi", "given": "Alessandro", "initials": "A", "orcid": "0000-0002-8773-9144", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/561898f673ca449ebe79aef02751e921.json"}}, {"family": "Rule", "given": "Jody A", "initials": "JA", "orcid": "0000-0002-4019-372X", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/2779649f7aac411a9b8eb25b0ed9a934.json"}}, {"family": "Lee", "given": "William M", "initials": "WM", "orcid": "0000-0002-2783-5441", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/0daeba0f2f7f497aa8defa1f568187ee.json"}}, {"family": "Landegren", "given": "Nils", "initials": "N", "orcid": "0000-0002-6163-9540", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/6765f1617f51434baf93e4683965f03a.json"}}, {"family": "Cobat", "given": "Aur\u00e9lie", "initials": "A", "orcid": "0000-0001-7209-6257", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/55186a4757e7417389f415061b59ab5c.json"}}, {"family": "Casanova", "given": "Jean-Laurent", "initials": "JL", "orcid": "0000-0002-7782-4169", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/264335517ad94440b23a027fe66f767d.json"}}, {"family": "Jouanguy", "given": "Emmanuelle", "initials": "E", "orcid": "0000-0002-7358-9157", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/de52d6ee013c4d18888fed695f547f54.json"}}], "type": "journal article", "published": "2026-03-02", "journal": {"title": "J. Exp. Med.", "issn": "1540-9538", "volume": "223", "issue": "3", "issn-l": "0022-1007"}, "abstract": "Fulminant viral hepatitis (FVH) is a devastating condition caused by hepatotropic viruses such as hepatitis A virus (HAV), hepatitis B virus (HBV), and HSV-1/2. We studied 149 FVH patients (73 males and 76 females, aged 1-76) for blood autoantibodies (auto-Abs) neutralizing type I interferons (IFNs; IFN-\u03b12, -\u03b2, -\u03c9). Six of 16 (37.5%) HSV-triggered FVH patients carried such auto-Abs on admission, including three with a previously known autoimmune disease. These patients contrasted with 133 HAV- (n = 46) or HBV-triggered (n = 87) patients, none of whom had such detectable auto-Abs. Odds ratios for HSV-triggered FVH in individuals with auto-Abs ranged from 35.3 (95% CI: 13.0-96.2; P < 10-7) for those neutralizing only 100 pg/ml IFN-\u03b1/\u03c9 to 1,895 (CI: 448.5-8,002; P < 10-12) for those neutralizing both IFN-\u03b1 and IFN-\u03c9 at 10 ng/ml. Over one third of HSV-triggered FVH cases in this international cohort were due to preexisting auto-Abs. This finding highlights auto-Abs against type I IFNs as a major determinant of HSV-FVH and paves the way for targeted preventive or therapeutic interventions.", "doi": "10.1084/jem.20251760", "pmid": "41348319", "labels": [], "xrefs": [{"db": "pmc", "key": "PMC12679989"}, {"db": "pii", "key": "278554"}], "notes": [], "created": "2026-08-20T09:38:20.673Z", "modified": "2026-08-20T09:38:22.004Z"}, {"entity": "publication", "iuid": "126db5b80e85485bb82fde8653bdb058", "links": {"self": {"href": "https://publications-affiliated.scilifelab.se/publication/126db5b80e85485bb82fde8653bdb058.json"}, "display": {"href": "https://publications-affiliated.scilifelab.se/publication/126db5b80e85485bb82fde8653bdb058"}}, "title": "The immunopathological landscape of human pre-TCR\u03b1 deficiency: From rare to common variants.", "authors": [{"family": "Materna", "given": "Marie", "initials": "M", "orcid": "0000-0003-2145-5285", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/453f535bbfd04105ad4e36fa8082b65a.json"}}, {"family": "Delmonte", "given": "Ottavia M", "initials": "OM", "orcid": "0000-0002-4772-0799", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/b43eba3fcbc045a3ae9d4a643c1dbb8e.json"}}, {"family": "Bosticardo", "given": "Marita", "initials": "M", "orcid": "0000-0002-1771-4523", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/6ff284bc536745cea923d424cbccb549.json"}}, {"family": "Momenilandi", "given": "Mana", "initials": "M", "orcid": "0000-0002-5340-2762", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/9601ab27fab34baaaafd25d43572b239.json"}}, {"family": "Conrey", "given": "Peyton E", "initials": "PE", "orcid": "0000-0001-7766-9878", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/84bb121e6f6648e5bf7bed6750d4db5e.json"}}, {"family": "Charmeteau-De Muylder", "given": "B\u00e9n\u00e9dicte", "initials": "B"}, {"family": "Bravetti", "given": "Clotilde", "initials": "C"}, {"family": "Bellworthy", "given": "Rebecca", "initials": "R", "orcid": "0009-0002-5878-1527", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/8a0997ac5da249b0b795ae7bc269860d.json"}}, {"family": "Cederholm", "given": "Axel", "initials": "A", "orcid": "0009-0006-5973-9637", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/f21e794263354dd19a65d1aa0871a194.json"}}, {"family": "Staels", "given": "Frederik", "initials": "F"}, {"family": "Ganoza", "given": "Christian A", "initials": "CA", "orcid": "0000-0002-5891-5372", "researcher": {"href": "https://publications-affiliated.scilifelab.se/researcher/acfca3c91f4a4e7ba223156857313621.json"}}, {"family": "Darko", "given": "Samuel", "initials": "S", "orcid": "0000-0002-9711-8122", "researcher": {"href": 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"published": "2024-03-00", "journal": {"title": "Science (New York, N.Y.)", "issn": "1095-9203", "volume": "383", "issue": "6686", "pages": "eadh4059", "issn-l": "0036-8075"}, "abstract": "We describe humans with rare biallelic loss-of-function PTCRA variants impairing pre-\u03b1 T cell receptor (pre-TCR\u03b1) expression. Low circulating naive \u03b1\u03b2 T cell counts at birth persisted over time, with normal memory \u03b1\u03b2 and high \u03b3\u03b4 T cell counts. Their TCR\u03b1 repertoire was biased, which suggests that noncanonical thymic differentiation pathways can rescue \u03b1\u03b2 T cell development. Only a minority of these individuals were sick, with infection, lymphoproliferation, and/or autoimmunity. We also report that 1 in 4000 individuals from the Middle East and South Asia are homozygous for a common hypomorphic PTCRA variant. They had normal circulating naive \u03b1\u03b2 T cell counts but high \u03b3\u03b4 T cell counts. Although residual pre-TCR\u03b1 expression drove the differentiation of more \u03b1\u03b2 T cells, autoimmune conditions were more frequent in these patients compared with the general population.", "doi": "10.1126/science.adh4059", "pmid": "38422122", "labels": [], "xrefs": [{"db": "mid", "key": "NIHMS1972302"}, {"db": "pmc", "key": "PMC10958617"}], "notes": [], "created": "2026-08-20T11:59:36.719Z", "modified": "2026-08-20T11:59:39.340Z"}]}