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Inflammation and host-pathogen interaction: Cause and consequence in cystic fibrosis lung disease

Journal

JOURNAL OF CYSTIC FIBROSIS
Volume 17, Issue 2, Pages S40-S45

Publisher

ELSEVIER SCIENCE BV
DOI: 10.1016/j.jcf.2017.10.004

Keywords

Lung; Host defence; Inflammation; Animal models; Infection; Cystic fibrosis

Funding

  1. Dutch Cystic Fibrosis Foundation NCFS [MV HIT-CFI-C7]
  2. EU ERARE-INSTINCT, NIH [5R01HL126603-03]
  3. ZONMW [435000035]
  4. Lung Foundation Netherlands [3.3.10.027]
  5. Italian CF research Foundation [9/2014, 15/2016]
  6. SYSGENET network [BM0901]
  7. European Respiratory Society Fellowship [STRTF 2014-5493]
  8. Cystic Fibrosis Foundation [WELDON15G0]
  9. European Union Seventh Framework Program (FP7) [603038]
  10. Lexicon Inc (CA, MA USA)

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Cystic Fibrosis (CF) lung disease is associated with dysregulation of host defence systems, which ultimately disrupts the balance between inflammation and resolution and leaves the host susceptible to repeated infection. However, the mechanisms underlying these defects are complex and continue to garner significant interest among the CF research community. This review explores emerging data on novel aspects of innate host defence with promising biomarker and therapeutic potential for CF lung disease. Improved understanding of inflammation and host defence against pathogens in patients and animal models during the progression of CF lung disease is pivotal for the discovery of new therapeutics that can limit and/or prevent damage from birth. (C) 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

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