Neutralization of IFNg defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice
نویسندگان
چکیده
(1) Institut National de la Santé et de la Recherche M Laboratoire du Développement Normal et Patho Immunitaire, Paris, France. (2) Université Paris Descartes, Faculté de Médecine Descartes, Institut Fédératif de Recherche Neck (IFR94), Paris, France. (3) Assistance Publique-Hôpitaux de Paris, Hôpital Nec Unité d’Immunologie et Hématologie Pédiatrique, Pa (4) Assistance Publique-Hôpitaux de Paris, Hôpital He pathologie, Département de Pathologie, Créteil, Fran (5) Assistance Publique-Hôpitaux de Paris, Hôpital Nec Service d’Anatomie et de Cytologie Pathologiques, Pa (6) NovImmune SA, Geneva, Switzerland. (7) Institut National de la Santé et de la Recherche M Necker-Enfants Malades, Faculté de Médecine d Descartes, Paris, France. *Corresponding authors: Tel: þ33 1 44 49 50 08; Fax: þ33 1 42 73 06 40 E-mails: [email protected]; genevieve.de-saint
منابع مشابه
Neutralization of IFNγ defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice
Hereditary haemophagocytic lymphohistiocytosis (HLH) is a fatal inflammatory disease and treatments currently may lead to serious side effects. There is a pressing need for effective, less toxic treatments for this disease. Previous reports have suggested that interferon gamma (IFNgamma) has a role in the pathogenesis of HLH. Here, we report that blocking IFNgamma had a therapeutic effect in tw...
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Inherited defects of granule-dependent cytotoxicity led to the life-threatening immune disorder hemophagocytic lymphohistiocytosis (HLH), characterized by uncontrolled CD8 T-cell and macrophage activation. In a cohort of HLH patients with genetic abnormalities expected to result in the complete absence of perforin, Rab27a, or syntaxin-11, we found that disease severity as determined by age at H...
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