Toll-like receptors as therapeutic targets in cystic fibrosis

Abstract

Background: Toll-like receptors (TLRs) are pattern recognition receptors that act as a first-line of defence in the innate immune response by recognising and responding to conserved molecular patterns in microbial factors and endogenous danger signals. Cystic fibrosis (CF)-affected airways represent a milieu potentially rich in TLR agonists and the chronic inflammatory phenotype evident in CF airway epithelial cells is probably due in large part to activation of TLRs. Objective/methods: To examine the prospects of developing novel therapies for CF by targeting TLRs. We outline the expression and function of TLRs and explore the therapeutic potential of naturally-occurring and synthetic TLR inhibitors for CF. Results/conclusion: Modulation of TLRs has therapeutic potential for the inflammatory lung manifestations of CF.

Keywords::

• cystic fibrosis
• inflammation
• innate immunity
• therapeutics
• toll-like receptors

Acknowledgements

Cystic fibrosis research in this laboratory is generously funded by the Programme for Research in Third level Institutes, the Health Research Board, the CF Hope Source, the CF Association of Ireland and Molecular Medicine Ireland.

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