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Balancing the immune response in cystic fibrosis: using zebrafish models of infection and inflammation to uncover new therapeutic approaches

Total Cost €


EC-Contrib. €






 CFZEBRA project word cloud

Explore the words cloud of the CFZEBRA project. It provides you a very rough idea of what is the project "CFZEBRA" about.

quality    regulating    regulator    microenvironment    improvement    defects    expectancy    immune    found    limited    fellowship    pathogens    active    pathophysiology    techniques    dominated    disorder    tractable    persistent    cftr    progressive    unmet    suggest    injury    expression    molecules    fibrosis    abnormalities    cystic    neutrophil    susceptibility    mortality    genetic    model    mucociliary    conductance    models    therapeutic    guide    patients    suitable    confirm    physiological    imaging    functions    deficient    cell    pathology    severe    primary    morbidity    larval    mutations    therapies    function    macrophages    causes    innate    human    deficiency    cf    animal    inflammatory    benefit    infections    inflammation    zebrafish    chronic    consequent    lines    larvae    pulmonary    innovative    correcting    limiting    caused    gives    infection    gene    transmembrane    infective    elucidate    accompanied    recapitulate    disease    context    alterations    dynamic    life    data    balance    combined    lung    lack    preliminary   

Project "CFZEBRA" data sheet

The following table provides information about the project.


Organization address
postcode: S10 2TN

contact info
title: n.a.
name: n.a.
surname: n.a.
function: n.a.
email: n.a.
telephone: n.a.
fax: n.a.

 Coordinator Country United Kingdom [UK]
 Total cost 195˙454 €
 EC max contribution 195˙454 € (100%)
 Programme 1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
 Code Call H2020-MSCA-IF-2016
 Funding Scheme MSCA-IF-EF-ST
 Starting year 2017
 Duration (year-month-day) from 2017-06-01   to  2019-05-31


Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    THE UNIVERSITY OF SHEFFIELD UK (SHEFFIELD) coordinator 195˙454.00


 Project objective

Cystic fibrosis (CF) is a life-limiting disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Chronic pulmonary infections accompanied by persistent neutrophil-dominated inflammation result in severe progressive lung injury and are the leading causes of morbidity and mortality of CF patients. In addition to known mucociliary defects, several lines of evidence suggest primary alterations in innate immune responses contribute to CF pathology. Currently, our understanding is limited by the lack of suitable animal models that recapitulate immune abnormalities found in CF patients. To address these unmet needs, I propose to develop zebrafish larvae as a tractable animal model to investigate the role of CFTR in regulating inflammation and infection. In preliminary data, reduced zebrafish cftr expression is characterized by more inflammation and increased susceptibility to infection with CF pathogens. In this fellowship, I will use this innovative model to recapitulate aspects of the CF microenvironment. Using innovative genetic techniques combined with dynamic imaging, I will elucidate physiological functions of CFTR in innate immune pathophysiology in CF and identify new therapeutic molecules active in a CFTR-deficient context to balance infection and inflammation to benefit patients with CF. Finally, I will confirm my findings in human CF macrophages. Using larval zebrafish gives a unique insight into immune cell function in CFTR deficiency. This will help guide future therapies aimed at correcting the innate susceptibility of CF patients to infective and inflammatory lung disease, with consequent improvement of their life quality and expectancy.


year authors and title journal last update
List of publications.
2018 A. Bernut, A. Floto, S. Renshaw
WS04.6 Balancing the immune response in cystic fibrosis: using zebrafish models of inflammation to uncover new therapeutic approaches
published pages: S8-S9, ISSN: 1569-1993, DOI: 10.1016/s1569-1993(18)30142-5
Journal of Cystic Fibrosis 17 2019-11-08
2019 Audrey Bernut, Christian Dupont, Nikolay V. Ogryzko, Aymeric Neyret, Jean-Louis Herrmann, R. Andres Floto, Stephen A. Renshaw, Laurent Kremer
CFTR Protects against Mycobacterium abscessus Infection by Fine-Tuning Host Oxidative Defenses
published pages: 1828-1840.e4, ISSN: 2211-1247, DOI: 10.1016/j.celrep.2019.01.071
Cell Reports 26/7 2019-11-08

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