European Cystic Fibrosis Society

The aim of the ECFS Basic Science meetings is to help foster and initiate new research towards a better understanding of the pathogenesis of CF.Basic research will ultimately contribute to new and improved therapies for CF. This year’s meeting was chaired by J.P. Clancy (University of Alabama, Birmingham, USA), Michael Gray (Newcastle University, UK) and Harry Cuppens (KULeuven, Leuven, Belgium).

A key characteristic of the conference is the high attendance of young participants, both PhD students and post-docs, who contribute enormously to the success of this annual event with their enthusiasm and vivid discussions.

The conference attracted a record number of participants (113) from 14 European and North American countries. This year’s programme included two key-note lectures and nine symposia with international experts covering topical aspects of basic research in cystic fibrosis together with invited talks from submitted abstracts. There were also four informal interactive special interest group discussions and two evening poster sessions (48 abstracts).

Speakers discussed a variety of hot topics of basic research into cystic fibrosis, with many presenting unpublished data which greatly added to the immediacy and exciting nature of the meeting.

The first key-note speaker, Hugo De Jonge, opened the meeting and took us on a journey of CFTR in the gastrointestinal tract in CF patients and CF mice.

Topics of the symposia and lectures ranged from new insights into the 3D structure of CFTR, its regulation by phosphorylation, to its folding, processing and degradation. Also, attention was given to how to repair the CFTR folding defects caused by CFTR mutations.

Apart from CFTR, there was also much interest in other channels, such as Anoctamins, Slc26 anion transporters and ENaC.
One symposium dealt with mucus biogenesis and disruption in CF. Very ‘video-entertaining’ was the lecture by S. Rowe, who presented a new assay for modulation of ion transport and mucus clearance in cells and tissues by video-rate ultrahigh-resolution 3D optical imaging by spectral domain optical coherence tornography.

Much attention was also given to inflammatory signalling in CF lung disease and the CF pulmonary microbiome. Here, topics included the CFTR interactome in the inflammation process, and the clonal variation of Pseudomonas aeruginosa isolates from CF airways during the last 20 years.

We also heard about the latest development of lung disease in two distinct transgenic animal models. The long chicken/egg dilemma of ‘what comes first, infection or inflammation’ may now be (started to be) resolved. In the CF pig model, no evidence was found yet that inflammation precedes infection in the CF lung. Also, the findings of a new model, the CFTR knockout ferret, were presented for the first time. Of much interest was the comparison of all the CF models obtained so far, and CF in humans, with respect to the degree, extent and time of disease involved in the different affected organs in the different CF models. Clearly, these animal models are going to be invaluable for a better understanding of the progression of airway disease, as well as for the development of improved therapies for CF.

In the genetic symposium, the latest findings of the North-American and Canadian CF modifier studies were reported. Also the CFTR2 project was presented, in which 96-98% of all the 1600 different reported mutations and variations in the CFTR gene will be characterised at the clinical and functional level, which will be an invaluable tool in future CF diagnostics.

The final symposium was a dedicated symposium on translating basic research into clinical practice. Here projects were presented that use high throughput screening of small molecules, and siRNA screens, for identifying targets that affect CFTR, F508del CFTR, CaCC and ENaC. The ultimate goal is the finding of new modulators for treatment of CF disease. There is no doubt that major advances in treatment of CF are going to rely heavily on these new tools.

Much fun were the oral flash presentations of the posters by the young PhD students and postdocs. Here, young scientists got one minute and one slide to summarise their poster and attract the participants of the meeting to their poster. I must say, although they had only one minute, overall they sticked better to their allocated time than some senior scientists during the regular presentations. The quality of the work presented was extremely high.

This year, the best poster prizes went to Mairead Kelly (UK), Julia Dürr (Germany) and Alexandre Hinzpeter (France). Congratulations! These awards were presented during the closing dinner of the meeting. Even more memorable at this closing social event was the karaoke performance by my co-chair JP Clancy, who gave an awesome imitation of Mick Jagger. 

The attendance of young researchers was greatly helped by substantial travel awards from Novartis and the Cystic Fibrosis Trust. Other sponsors were Bayer HealthCare, Innogenetics and Casen Fleet Laboratories. The Physiological Society and Transave Inhalation Biotherapeutics provided educational grants. We would like to thank them all for their continuing support of this meeting. Finally, we would like to express our gratitude to the ECFS and the Cystic Fibrosis Foundation on both sides of the Atlantic who supported the conference. Without this support these meetings would not be possible.

This meeting clearly demonstrated the need of basic research into CF, and we look forward to hearing more exciting news in 2011.

Harry Cuppens, KULeuven, Leuven, Belgium