| First Author | Svedin E | Year | 2017 |
| Journal | J Infect Dis | Volume | 216 |
| Issue | 10 | Pages | 1308-1317 |
| PubMed ID | 28968805 | Mgi Jnum | J:272755 |
| Mgi Id | MGI:6282060 | Doi | 10.1093/infdis/jix474 |
| Citation | Svedin E, et al. (2017) A Link Between a Common Mutation in CFTR and Impaired Innate and Adaptive Viral Defense. J Infect Dis 216(10):1308-1317 |
| abstractText | Acute respiratory virus infections predispose the cystic fibrosis (CF) lung to chronic bacterial colonization, which contributes to high mortality. For reasons unknown, respiratory virus infections have a prolonged duration in CF. Here, we demonstrate that mice carrying the most frequent cystic fibrosis transmembrane conductance regulator (CFTR) mutation in humans, DeltaF508, show increased morbidity and mortality following infection with a common human enterovirus. DeltaF508 mice demonstrated impaired viral clearance, a slower type I interferon response and delayed production of virus-neutralizing antibodies. While the DeltaF508 mice had a normal immune cell repertoire, unchanged serum immunoglobulin concentrations and an intact immune response to a T-cell-independent antigen, their response to a T-cell-dependent antigen was significantly delayed. Our studies reveal a novel function for CFTR in antiviral immunity and demonstrate that the DeltaF508 mutation in cftr is coupled to an impaired adaptive immune response. This important insight could open up new approaches for patient care and treatment. |
