期刊
SCIENCE
卷 348, 期 6233, 页码 448-453出版社
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/science.aaa1578
关键词
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资金
- National Center for Research Resources
- National Center for Advancing Translational Sciences [8UL1TR000043]
- NIH [5R01NS072381, 5R01AI100887, 1U19AI109945]
- Rockefeller University
- St. Giles Foundation
- French National Research Agency under the Investments for the Future program [ANR-10-IAHU-01]
- Laboratoire d'Excellence Integrative Biology of Emerging Infectious Diseases [ANR-10-LABX-62-IBEID]
- INSERM
- Paris Descartes University
- ERC [2010-StG-261299]
- Center for Research on Influenza Pathogenesis
- NIAID [HHSN272201400008C]
- [SRP055919]
- Grants-in-Aid for Scientific Research [25713039, 25670477] Funding Source: KAKEN
- Biotechnology and Biological Sciences Research Council [1168004] Funding Source: researchfish
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.
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