Peer Reviewed

1

Document Type

Article

Publication Date

1-9-2012

Keywords

Adaptive Immunity, Chemokines, Cystic Fibrosis, Fibrosis, Host-Pathogen Interactions, Humans, Immune System, Immunity, Innate, Inflammation, Pseudomonas Infections, Pseudomonas aeruginosa, Respiratory System, Respiratory Tract Infections, Toll-Like Receptors

Funder/Sponsor

NIH (HL102371-01A1). CF Foundation (GAGGAR07A0). DFG (MA 2081/3-3 and MA 2081/4-; Emmy Noether Programme HA 5274/3-1) BMBF (DZL).

Comments

The original article is available at https://www.sciencedirect.com

Abstract

Chronic lung disease determines the morbidity and mortality of cystic fibrosis (CF) patients. The pulmonary immune response in CF is characterized by an early and non-resolving activation of the innate immune system, which is dysregulated at several levels. Here we provide a comprehensive overview of innate immunity in CF lung disease, involving (i) epithelial dysfunction, (ii) pathogen sensing, (iii) leukocyte recruitment, (iv) phagocyte impairment, (v) mechanisms linking innate and adaptive immunity and (iv) the potential clinical relevance. Dissecting the complex network of innate immune regulation and associated pro-inflammatory cascades in CF lung disease may pave the way for novel immune-targeted therapies in CF and other chronic infective lung diseases.

Disciplines

Medicine and Health Sciences

Citation

Hartl D, Gaggar A, Bruscia E, Hector A, Marcos V, Jung A, Greene C, McElvaney G, Mall M, Döring G. Innate immunity in cystic fibrosis lung disease. Journal of Cystic Fibrosis. 2012;11(5):363-82.

PubMed ID

22917571

DOI Link

10.1016/j.jcf.2012.07.003

Creative Commons License

Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-Share Alike 4.0 License.

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