Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages
Abstract
:1. A Brief History
2. The Host and the Environment: Inflammation and Infection (and More Inflammation)
3. The Evolutionary Past (Last 5000–50 Years)
4. The Long Now (Last 50 Years)
5. The Present Moment (Last 5 Years)
6. The Near Future (Next 5 Years)
7. Future Horizons
Author Contributions
Funding
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Traditional Class | CFTR Impairment | Mutations Example | Frequency of Occurrence α | CFTR Modulator Treatment |
---|---|---|---|---|
I | No mRNA/functional protein | G542X | 22% | None |
II | No protein trafficking | F508Del | 88% | Lumacaftor–ivacaftor, tezacaftor–ivacaftor, or elexacaftor–tezacaftor–ivacaftor |
III | Abnormal gating of channels | G551D | 6% | Ivacaftor |
IV | Reduced channel conductance | R117H | 6% | Ivacaftor in select mutations |
V | Decreased protein synthesis | A455E | 5% | Tezacaftor–ivacaftor in select mutations |
VI | Reduced protein stability | c.120del23 | 5% | Tezacaftor–ivacaftor in select mutations |
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Yu, C.; Kotsimbos, T. Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages. Int. J. Mol. Sci. 2023, 24, 4052. https://doi.org/10.3390/ijms24044052
Yu C, Kotsimbos T. Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages. International Journal of Molecular Sciences. 2023; 24(4):4052. https://doi.org/10.3390/ijms24044052
Chicago/Turabian StyleYu, Christiaan, and Tom Kotsimbos. 2023. "Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages" International Journal of Molecular Sciences 24, no. 4: 4052. https://doi.org/10.3390/ijms24044052