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Differential Expression of Interferon-Alpha Protein Provides Clues to Tissue Specificity Across Type I Interferonopathies.
Lodi, Lorenzo; Melki, Isabelle; Bondet, Vincent; Seabra, Luis; Rice, Gillian I; Carter, Edwin; Lepelley, Alice; Martin-Niclós, Maria José; Al Adba, Buthaina; Bader-Meunier, Brigitte; Barth, Magalie; Blauwblomme, Thomas; Bodemer, Christine; Boespflug-Tanguy, Odile; Dale, Russel C; Desguerre, Isabelle; Ducrocq, Camille; Dulieu, Fabienne; Dumaine, Cécile; Ellul, Pierre; Hadchouel, Alice; Hentgen, Véronique; Hié, Miguel; Hully, Marie; Jeziorski, Eric; Lévy, Romain; Mochel, Fanny; Orcesi, Simona; Passemard, Sandrine; Pouletty, Marie; Quartier, Pierre; Renaldo, Florence; Seidl, Rainer; Shetty, Jay; Neven, Bénédicte; Blanche, Stéphane; Duffy, Darragh; Crow, Yanick J; Frémond, Marie-Louise.
Afiliación
  • Lodi L; Université de Paris, Imagine Institute, Laboratory of Neurogenetics and Neuroinflammation, 24 boulevard du Montparnasse, 75015, Paris, France.
  • Melki I; Department of Health Sciences, University of Florence - Meyer Children's University Hospital, Florence, Italy.
  • Bondet V; Université de Paris, Imagine Institute, Laboratory of Neurogenetics and Neuroinflammation, 24 boulevard du Montparnasse, 75015, Paris, France.
  • Seabra L; General Paediatrics- Infectious Diseases and Internal Medicine Department, Robert-Debré Hospital, AP-HP, Nord - Université de Paris, Paris, France.
  • Rice GI; Paediatric Haematology-Immunology and Rheumatology Unit, Necker Hospital, AP-HP, Centre - Université de Paris, Paris, France.
  • Carter E; Translational Immunology Lab, Institut Pasteur, Paris, France.
  • Lepelley A; Université de Paris, Imagine Institute, Laboratory of Neurogenetics and Neuroinflammation, 24 boulevard du Montparnasse, 75015, Paris, France.
  • Martin-Niclós MJ; School of Biological Sciences, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, Division of Evolution and Genomic Sciences, University of Manchester, Manchester, UK.
  • Al Adba B; Centre for Genomic and Experimental Medicine, Medical Research Council Institute of Genetics and Molecular Medicine, The University of Edinburgh, Crewe Road, Edinburgh, EH4 2XU, UK.
  • Bader-Meunier B; Université de Paris, Imagine Institute, Laboratory of Neurogenetics and Neuroinflammation, 24 boulevard du Montparnasse, 75015, Paris, France.
  • Barth M; Université de Paris, Imagine Institute, Laboratory of Neurogenetics and Neuroinflammation, 24 boulevard du Montparnasse, 75015, Paris, France.
  • Blauwblomme T; Department of Paediatric Rheumatology, Sidra Medicine, Doha, Qatar.
  • Bodemer C; Paediatric Haematology-Immunology and Rheumatology Unit, Necker Hospital, AP-HP, Centre - Université de Paris, Paris, France.
  • Boespflug-Tanguy O; UMR CNRS 6015-INSERM U1083, MitoVasc Institute, University of Angers, Angers, France.
  • Dale RC; Paediatric Neurosurgery Unit, Necker Hospital, AP-HP, Centre Université de Paris, Paris, France.
  • Desguerre I; Paediatric Dermatology Department, Necker Hospital, AP-HP, Centre Université de Paris, Paris, France.
  • Ducrocq C; Paediatric Neurology Department, Robert-Debré Hospital, AP-HP, Nord - Université de Paris, Paris, France.
  • Dulieu F; Kids Neuroscience Centre, The Children's Hospital at Westmead, University of Sydney, Westmead, NSW, Australia.
  • Dumaine C; Paediatric Neurology Department, Necker Hospital, AP-HP, Centre Université de Paris, Paris, France.
  • Ellul P; General Paediatrics- Infectious Diseases and Internal Medicine Department, Robert-Debré Hospital, AP-HP, Nord - Université de Paris, Paris, France.
  • Hadchouel A; Paediatrics Department, Nice Hospitals, CHU LENVAL, Nice, France.
  • Hentgen V; General Paediatrics- Infectious Diseases and Internal Medicine Department, Robert-Debré Hospital, AP-HP, Nord - Université de Paris, Paris, France.
  • Hié M; Department of Child and Adolescent Psychiatry, Robert-Debré Hospital, AP-HP, Nord - Université de Paris, Paris, France.
  • Hully M; INSERM, Immunology-Immunopathology-Immunotherapy (i3), Sorbonne Université, Paris, France.
  • Jeziorski E; Paediatric Pulmonology Department, Necker Hospital, AP-HP, Centre Université de Paris, Paris, France.
  • Lévy R; Paediatrics Department, Versailles Hospital, Le Chesnay, France.
  • Mochel F; French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne Université, Pa
  • Orcesi S; Paediatric Neurology Department, Necker Hospital, AP-HP, Centre Université de Paris, Paris, France.
  • Passemard S; Paediatrics Department, Centre Hospitalier Universitaire de Montpellier, Montpellier, France.
  • Pouletty M; Paediatric Haematology-Immunology and Rheumatology Unit, Necker Hospital, AP-HP, Centre - Université de Paris, Paris, France.
  • Quartier P; National Reference Center for Neurometabolic Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne Université, Paris, France.
  • Renaldo F; Institut du Cerveau et de la Moelle épinière, INSERM U 1127, Sorbonne Université, Paris, France.
  • Seidl R; Child Neurology and Psychiatry Unit IRCCS Mondino Foundation, Pavia, Italy.
  • Shetty J; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.
  • Neven B; Paediatric Neurology Department, Robert-Debré Hospital, AP-HP, Nord - Université de Paris, Paris, France.
  • Blanche S; General Paediatrics- Infectious Diseases and Internal Medicine Department, Robert-Debré Hospital, AP-HP, Nord - Université de Paris, Paris, France.
  • Duffy D; Paediatric Haematology-Immunology and Rheumatology Unit, Necker Hospital, AP-HP, Centre - Université de Paris, Paris, France.
  • Crow YJ; Paediatric Neurology Department, Trousseau Hospital, AP-HP, Sorbonne Université, Paris, France.
  • Frémond ML; Department of Paediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.
J Clin Immunol ; 41(3): 603-609, 2021 04.
Article en En | MEDLINE | ID: mdl-33411153
Whilst upregulation of type I interferon (IFN) signaling is common across the type I interferonopathies (T1Is), central nervous system (CNS) involvement varies between these disorders, the basis of which remains unclear. We collected cerebrospinal fluid (CSF) and serum from patients with Aicardi-Goutières syndrome (AGS), STING-associated vasculopathy with onset in infancy (SAVI), presumed monogenic T1Is (pT1I), childhood systemic lupus erythematosus with neuropsychiatric features (nSLE), non-IFN-related autoinflammation (AI) and non-inflammatory hydrocephalus (as controls). We measured IFN-alpha protein using digital ELISA. Eighty-two and 63 measurements were recorded respectively in CSF and serum of 42 patients and 6 controls. In an intergroup comparison (taking one sample per individual), median CSF IFN-alpha levels were elevated in AGS, SAVI, pT1I, and nSLE compared to AI and controls, with levels highest in AGS compared to all other groups. In AGS, CSF IFN-alpha concentrations were higher than in paired serum samples. In contrast, serum IFN was consistently higher compared to CSF levels in SAVI, pT1I, and nSLE. Whilst IFN-alpha is present in the CSF and serum of all IFN-related diseases studied here, our data suggest the primary sites of IFN production in the monogenic T1I AGS and SAVI are, respectively, the CNS and the periphery. These results inform the diagnosis of, and future therapeutic approaches to, monogenic and multifactorial T1Is.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Especificidad de Órganos / Interferón Tipo I / Regulación de la Expresión Génica / Interferón-alfa / Susceptibilidad a Enfermedades Tipo de estudio: Observational_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: J Clin Immunol Año: 2021 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Países Bajos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Especificidad de Órganos / Interferón Tipo I / Regulación de la Expresión Génica / Interferón-alfa / Susceptibilidad a Enfermedades Tipo de estudio: Observational_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: J Clin Immunol Año: 2021 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Países Bajos