The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency
Open accessAbstract
Life-threatening pulmonary influenza can be caused by inborn errors of type I and III IFN immunity. We report a 5-yr-old child with severe pulmonary influenza at 2 yr. She is homozygous for a loss-of-function IRF9 allele. Her cells activate gamma-activated factor (GAF) STAT1 homodimers but not IFN-stimulated gene factor 3 (ISGF3) trimers (STAT1/STAT2/IRF9) in response to IFN-α2b. The transcriptome induced by IFN-α2b in the patient’s cells is much narrower than that of control cells; however, induction of a subset of IFN-stimulated gene transcripts remains detectable. In vitro, the patient’s cells do not control three respiratory viruses, influenza A virus (IAV), parainfluenza virus (PIV), and respiratory syncytial virus (RSV). These phenotypes are rescued by wild-type IRF9, whereas silencing IRF9 expression in control cells increases viral replication. However, the child has controlled various common viruses in vivo, including respiratory viruses other than IAV. Our findings show that human IRF9- and ISGF3-dependent type I and III IFN responsive pathways are essential for controlling IAV.
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Cites
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“IRF9 also enables binding of the trimer to DNA, after its translocation to the nucleus (Veals et al., 1993; Darnell et al., 1994; Taniguchi et al., 2001; Stark and Darnell, 2012).”
Background1918 Influenza: the Mother of All Pandemics
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A loss-of-function IFNAR1 allele in Polynesia underlies severe viral diseases in homozygotes
Bastard, P., Hsiao, K.-C., Zhang, Q., Choin, J., Best, E., Chen, J., Gervais, A., Bizien, L., Materna, M., Harmant, C., et al. (2022) · Journal of Experimental Medicine · doi:10.1084/jem.20220028 · Cited by 70
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Metadata
- Authors
- Hernandez, N., Melki, I., Jing, H., Habib, T., Huang, S. S. Y., Danielson, J., Kula, T., Drutman, S., Belkaya, S., Rattina, V., Lorenzo-Diaz, L., Boulai, A., Rose, Y., Kitabayashi, N., Rodero, M. P., Dumaine, C., Blanche, S., Lebras, M.-N., Leung, M. C., Mathew, L. S., Boisson, B., Zhang, S.-Y., Boisson-Dupuis, S., Giliani, S., Chaussabel, D., Notarangelo, L. D., Elledge, S. J., Ciancanelli, M. J., Abel, L., Zhang, Q., Marr, N., Crow, Y. J., Su, H. C., & Casanova, J.-L.
- Venue
- Journal of Experimental Medicine
- Publisher
- Rockefeller University Press
- Published
- 2018-08-24
- Volume
- 215
- Issue
- 10
- Pages
- 2567-2585
- ISSN
- 0022-1007 (ISSN-L)
- 1540-9538
- OpenAlex
- W2888410625
- Cited by
- 210
- References
- 8
- FWCI
- 8.55
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Hernandez, N., Melki, I., Jing, H., Habib, T., Huang, S. S. Y., Danielson, J., Kula, T., Drutman, S., Belkaya, S., Rattina, V., Lorenzo-Diaz, L., Boulai, A., Rose, Y., Kitabayashi, N., Rodero, M. P., Dumaine, C., Blanche, S., Lebras, M.-N., Leung, M. C., . . . Casanova, J.-L. (2018). Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency. Journal of Experimental Medicine. https://doi.org/10.1084/jem.20180628
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
