Infectious disease. Life-threatening influenza and impaired interferon amplification in human IRF7 deficiency
| العنوان: | Infectious disease. Life-threatening influenza and impaired interferon amplification in human IRF7 deficiency |
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| المؤلفون: | Ciancanelli, Michael J., Huang, Sarah X. L., Luthra, Priya, Garner, Hannah, Itan, Yuval, Volpi, Stefano, Lafaille, Fabien G., Trouillet, Céline, Schmolke, Mirco, Albrecht, Randy A., Israelsson, Elisabeth, Lim, Hye Kyung, Casadio, Melina, Hermesh, Tamar, Lorenzo, Lazaro, Leung, Lawrence W., Pedergnana, Vincent, Boisson, Bertrand, Okada, Satoshi, Picard, Capucine, Ringuier, Benedicte, Troussier, Françoise, Chaussabel, Damien, Abel, Laurent, Pellier, Isabelle, Notarangelo, Luigi D., García-Sastre, Adolfo, Basler, Christopher F., Geissmann, Frédéric, Zhang, Shen-Ying, Snoeck, Hans-Willem, Casanova, Jean-Laurent |
| المساهمون: | St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller University [New York], Columbia University Medical Center (CUMC), Columbia University [New York], Icahn School of Medicine at Mount Sinai [New York] (MSSM), Centre for Cellular and Molecular Biology of Inflammation [London, UK] (CMCBI), King‘s College London, Division of Immunology and The Manton Center for Orphan Disease Research [Boston, MA, USA], Children's Hospital, Harvard Medical School, University of Genoa (UNIGE), Department of Systems Immunology [Seattle, WA, USA], Virginia Mason - Research Institute [Seattle, WA, USA], Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Service de chirurgie pédiatrique [CHU Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Service de Pédiatrie Générale [CHU Angers], Benaroya Research Institute [Seattle, WA, USA], Sidra Medical and Research Center [Doha, Qatar], Centre de Recherche en Cancérologie Nantes-Angers (CRCNA), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM)-Hôtel-Dieu de Nantes-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Laennec-Centre National de la Recherche Scientifique (CNRS)-Faculté de Médecine d'Angers-Centre hospitalier universitaire de Nantes (CHU Nantes), Service d'Immunologie Hématologie et Oncologie pédiatriques [CHU Angers], Service d'immuno-hématologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Supported by National Center for Research Resources and National Center for Advancing Translational Sciences grant 8UL1TR000043, NIH grants 5R01NS072381 (J.-L.C. and L.D.N.), 5R01AI100887 (L.D.N.), and 1U19AI109945 (C.F.B.), the Rockefeller University, the St. Giles Foundation, the French National Research Agency under the 'Investments for the Future' program grant ANR-10-IAHU-01, the Laboratoire d’Excellence Integrative Biology of Emerging Infectious Diseases (ANR-10-LABX-62-IBEID), INSERM, Paris Descartes University, and ERC grant 2010-StG‐261299 (F.G.), and Center for Research on Influenza Pathogenesis and the NIAID-funded Center of Excellence for Influenza Research and Surveillance contract HHSN272201400008C (A.G.-S.), ANR-10-IAHU-0001,Imagine,Institut Hospitalo-Universitaire Imagine(2010), ANR-10-LABX-0062,IBEID,Integrative Biology of Emerging Infectious Diseases(2010), Bernardo, Elizabeth, Instituts Hospitalo-Universitaires - Institut Hospitalo-Universitaire Imagine - - Imagine2010 - ANR-10-IAHU-0001 - IAHU - VALID, Integrative Biology of Emerging Infectious Diseases - - IBEID2010 - ANR-10-LABX-0062 - LABX - VALID, Università degli studi di Genova = University of Genoa (UniGe), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité) |
| المصدر: | Science Science, American Association for the Advancement of Science, 2015, 348 (6233), pp.448-53. ⟨10.1126/science.aaa1578⟩ Science, Vol. 348, No 6233 (2015) pp. 448-453 Science, 2015, 348 (6233), pp.448-53. ⟨10.1126/science.aaa1578⟩ |
| سنة النشر: | 2014 |
| مصطلحات موضوعية: | Heterozygote, Interferon Regulatory Factor-7, Induced Pluripotent Stem Cells, Influenza, Human/complications/genetics/immunology, [SDV.CAN]Life Sciences [q-bio]/Cancer, Genes, Recessive, Respiratory Mucosa, Article, Interferon Type I/biosynthesis/genetics, Influenza A Virus, H1N1 Subtype, [SDV.CAN] Life Sciences [q-bio]/Cancer, Fibroblasts/immunology, Influenza, Human, Leukocytes, Humans, Respiratory Distress Syndrome, Adult/genetics/immunology/virology, Leukocytes/immunology, Child, Interferon Regulatory Factor-7/genetics, Lung, Respiratory Mucosa/immunology, ddc:616, Respiratory Distress Syndrome, Induced Pluripotent Stem Cells/immunology, Dendritic Cells, Fibroblasts, Dendritic Cells/immunology, Lung/immunology, Interferon Type I, Mutation, Female |
| الوصف: | International audience; Severe influenza disease strikes otherwise healthy children and remains unexplained. We report compound heterozygous null mutations in IRF7, which encodes the transcription factor interferon regulatory factor 7, in an otherwise healthy child who suffered life-threatening influenza during primary infection. In response to influenza virus, the patient's leukocytes and plasmacytoid dendritic cells produced very little type I and III interferons (IFNs). Moreover, the patient's dermal fibroblasts and induced pluripotent stem cell (iPSC)-derived pulmonary epithelial cells produced reduced amounts of type I IFN and displayed increased influenza virus replication. These findings suggest that IRF7-dependent amplification of type I and III IFNs is required for protection against primary infection by influenza virus in humans. They also show that severe influenza may result from single-gene inborn errors of immunity. |
| وصف الملف: | application/pdf |
| تدمد: | 1095-9203 0036-8075 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::6ce1f38e10ed9e8095aaeaa4288f6f55 https://pubmed.ncbi.nlm.nih.gov/25814066 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.pmid.dedup....6ce1f38e10ed9e8095aaeaa4288f6f55 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 10959203 00368075 |
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