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Title

Infectious disease. Life-threatening influenza and impaired interferon amplification in human IRF7 deficiency

Authors
Ciancanelli, Michael J
Huang, Sarah X L
Luthra, Priya
Garner, Hannah
Itan, Yuval
Volpi, Stefano
Lafaille, Fabien G
Trouillet, Céline
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Published in Science. 2015, vol. 348, no. 6233, p. 448-453
Abstract 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.
Keywords ChildDendritic Cells/immunologyFemaleFibroblasts/immunologyGenes, RecessiveHeterozygoteHumansInduced Pluripotent Stem Cells/immunologyInfluenza A Virus, H1N1 SubtypeInfluenza, Human/complications/genetics/immunologyInterferon Regulatory Factor-7/geneticsInterferon Type I/biosynthesis/geneticsLeukocytes/immunologyLung/immunologyMutationRespiratory Distress Syndrome, Adult/genetics/immunology/virologyRespiratory Mucosa/immunology
Identifiers
PMID: 25814066
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Article (Published version) (1.9 MB) - document accessible for UNIGE members only Limited access to UNIGE
Structures
Research group Intéractions entre les pathogènes viraux et bactériens (947)
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(ISO format)
CIANCANELLI, Michael J et al. Infectious disease. Life-threatening influenza and impaired interferon amplification in human IRF7 deficiency. In: Science, 2015, vol. 348, n° 6233, p. 448-453. https://archive-ouverte.unige.ch/unige:76385

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Deposited on : 2015-10-22

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