Clinical MedicineImmunologyPulmonology Open Access | 10.1172/JCI161593

Carsten Schwarz,1 Patience Eschenhagen,1 Henrijette Schmidt,2,3 Thordis Hohnstein,4 Christina Iwert,5 Claudia Grehn,6 Jobst Roehmel,7 Eva Steinke,6,7,8 Mirjam Stahl,6,7,8 Laura Lozza,9 Ekaterina Tikhonova,2,3 Elisa Rosati,2,3 Ulrik Stervbo,10 Nina Babel,6,10 Jochen G. Mainz,11 Hilmar Wisplinghoff,12,13 Frank Ebel,14 Lei-Jie Jia,15 Matthew G. Blango,15 Peter Hortschansky,15 Sascha Brunke,16 Bernhard Hube,16,17 Axel A. Brakhage,15,17 Olaf Kniemeyer,15 Alexander Scheffold,3 and Petra Bacher2,3

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Schwarz, C. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Eschenhagen, P. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Schmidt, H. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Hohnstein, T. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Iwert, C. in: JCI | PubMed | Google Scholar |

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Grehn, C. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Roehmel, J. in: JCI | PubMed | Google Scholar |

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Steinke, E. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Stahl, M. in: JCI | PubMed | Google Scholar |

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Lozza, L. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Tikhonova, E. in: JCI | PubMed | Google Scholar |

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Rosati, E. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Stervbo, U. in: JCI | PubMed | Google Scholar |

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Babel, N. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Mainz, J. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt – Universität zu Berlin, Berlin, Germany.

8German Center for Lung Research (DZL), associated partner site, Berlin, Germany.

9Cell Biology Laboratory, Precision for Medicine GmbH, Berlin, Germany.

10Center for Translational Medicine and Immune Diagnostics Laboratory, Marien Hospital Herne, University Hospital of the Ruhr University Bochum, Herne, Germany.

11Brandenburg Medical School/Medizinische Hochschule Brandenburg (MHB), University, Pediatric Pulmonology/Cystic Fibrosis, Klinikum Westbrandenburg, Brandenburg an der Havel, Germany.

12Labor Dr. Wisplinghoff, Cologne, Germany.

13Institute for Virology and Microbiology, Witten/Herdecke University, Witten, Germany.

14Institute for Infectious Diseases and Zoonoses, LMU, Munich, Germany.

15Department of Molecular and Applied Microbiology, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

16Department of Microbial Pathogenicity Mechanisms, Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI), Jena, Germany.

17Institute of Microbiology, Friedrich Schiller University, Jena, Germany.

Address correspondence to: Petra Bacher, Christian-Albrechts Universität zu Kiel & Universitätsklinik Schleswig-Holstein, Institute of Immunology & Institute of Clinical Molecular Biology, Arnold-Heller-Str. 3, 24105 Kiel, Germany. Phone: 49.0.431.500.31005; Email: p.bacher@ikmb.uni-kiel.de.

Authorship note: CS and PE contributed equally to this work.

Find articles by Wisplinghoff, H. in: JCI | PubMed | Google Scholar

1Klinikum Westbrandenburg, Campus Potsdam, Cystic Fibrosis Section, Potsdam, Germany.

2Institute of Clinical Molecular Biology, Christian-Albrecht University of Kiel, Kiel, Germany.

3Institute of Immunology, Christian-Albrecht University of Kiel and UKSH Schleswig-Holstein, Kiel, Germany.

4Department of Microbiology, Infectious Diseases and Immunology, Charité – Universitätsmedizin Berlin, Berlin, Germany.

5Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Translational Immunology, Berlin, Germany.

6Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany.

7Department of Pediatric Respiratory Medicin