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Molecular Medicine Partnership Unit


Former MMPU Research Group Cystic Fibrosis

Marcus Mall and Carsten Schultz
Marcus Mall and Carsten Schultz

Marcus Mall and Carsten Schultz

MMPU Group 2006 - 2018
Project Summary

The focus of this project is on the pathogenesis and treatment of cystic fibrosis (CF) lung disease, one of the most common lethal hereditary diseases in Caucasians. We use an interdisciplinary approach combining chemical biology and in vivo analyses of genetically engineered mouse models to advance our current understanding of the pathogenesis, identify novel biomarkers and develop efficient therapeutic strategies for CF lung disease. The long term goal of this project is the translation of the results from pre-clinical models into the clinic.



Marcus Mall has studied the basic ion transport defect in cystic fibrosis (CF) airways and developed a transgenic mouse model of CF lung disease. Carsten Schultz developed small molecule compounds that target the CF ion transport defect, and fluorescent sensors to monitor enzyme activities and intracellular signaling events in living cells. They joined the MMPU in 2006 to coordinate their longstanding research interests in cystic fibrosis.


Important Publications

Chronic but not intermittent infection with Pseudomonas aeruginosa is associated with global changes of the lung microbiome in cystic fibrosis
Sébastien Boutin, Simon Y. Graeber, Mirjam Stahl, A. Susanne Dittrich, Marcus A. Mall, Alexander H. Dalpke
Eur Respir J. 2017 Oct 5;50(4). pii: 1701086. doi: 10.1183/13993003.01086-2017. Print 2017 Oct. PMID:28982777


Neutrophil elastase and matrix metalloproteinase 12 in cystic fibrosis lung disease.
Wagner CJ, Schultz C, Mall MA.
Mol Cell Pediatr. 2016 Dec;3(1):25. doi: 10.1186/s40348-016-0053-7. Epub 2016 Jul 25. Review


 Opens external link in new windowAirway Mucus Obstruction Triggers Macrophage Activation and MMP12-dependent Emphysema.
Trojanek JB, Cobos-Correa A, Diemer S, Kormann M, Schubert SC, Zhou-Suckow Z, Agrawal R, Duerr J, Wagner CJ, Schatterny J, Hirtz S, Sommerburg O, Hartl D, Schultz C, Mall MA.

Am J Respir Cell Mol Biol. 2014 Nov;51(5):709-20.


Opens external link in new windowA new player in the game: epithelial cathepsin S in early cystic fibrosis lung disease
Mall MA, Schultz C.
Am J Respir Crit Care Med. 2014 Jul 15;190(2):126-7. doi: 10.1164/rccm.201405-0935ED.


Opens external link in new windowLack of neutrophil elastase reduces inflammation, mucus hypersecretion and emphysema, but not mucus obstruction, in mice with CF-like lung disease.
Gehrig S, Duerr J, Weitnauer M, Wagner CJ, Graeber SY, Schatterny J, Hirtz S, Belaaouaj A, Dalpke AH, Schultz C, Mall MA.
Am J Respir Crit Care Med. 2014 Mar 28. [Epub ahead of print]

Opens external link in new windowFRET-based and other fluorescent proteinase probes.
Hu HY, Gehrig S, Reither G, Subramanian D, Mall MA, Plettenburg O, Schultz C.
Biotechnol J. 2014 Feb;9(2):266-81. doi: 10.1002/biot.201300201. Epub 2014 Jan 27.

Opens external link in new windowCFTR Regulates Early Pathogenesis of Chronic Obstructive Lung Disease in βENaC-Overexpressing Mice.
Johannesson B, Hirtz S, Schatterny J, Schultz C
, Mall MA.
PLoS One. 2012;7(8):e44059. Epub 2012 Aug 24


Opens external link in new windowmCLCA3 does not contribute to calcium-activated chloride conductance in murine airways. Mundhenk L, Johannesson B, Anagnostopoulou P, Braun J, Bothe MK, Schultz C, Mall MA, Gruber AD.
Am J Respir Cell Mol Biol. 2012 Jul;47(1):87-93. Epub 2012 Feb 23.


Opens external link in new windowSpatially resolved monitoring of neutrophil elastase activity with ratiometric fluorescent reporters.
Gehrig S, Mall MA, Schultz C.
Angew Chem Int Ed Engl. 2012 Jun 18;51(25):6258-61. doi: 10.1002/anie.201109226. Epub 2012 May 3.


Opens external link in new windowThe ßENaC-overexpressing mouse as a model of cystic fibrosis lung disease.
Zhou Z, Duerr J, Johannesson B, Schubert SC, Treis D, Harm M, Graeber SY, Dalpke A, Schultz C, Mall MA
J Cyst Fibros. 2011 Jun;10 Suppl 2:S172-82.


Opens external link in new windowAirway surface liquid volume regulation determines different airway phenotypes in liddle's compared to {beta}ENAC-overexpressing mice.
Mall MA, Button B, Johannesson B, Zhou Z, Livraghi A, Caldwell RA, Schubert SC, Schultz C, O'Neal WK, Pradervand S, Hummler E, Rossier BC, Grubb BR, Boucher RC.
J Biol Chem. 2010 Jun 21. 


Opens external link in new windowReporters to monitor cellular MMP12 activity
Amanda Cobos-Correa, Marcus A. Mall, Carsten Schultz
Proc. SPIE, Vol. 7576, 757605 (2010)


Opens external link in new windowMembrane-bound FRET probe visualizes MMP12 activity in pulmonary inflammation
Cobos-Correa A, Trojanek JB, Diemer S, Mall MA, Schultz C. 
Nat Chem Biol. 2009;5:628-30.  



Group Members

in alphabetical order

Raman Agrawal, PhD, Postdoc

Amanda Cobos-Correa, PhD

Stefanie Diemer, MD Student

Susanne Dittrich, MD, Physician Scientist

Dario Frey, PhD student (HBIGS)

Stefanie Gehrig, PhD Student, Postdoc

Matteo Guerra, PhD student (EMBL International PhD Programme - EIPP)

Nicole Heath, MD, Research Fellow

Stephanie Hirtz, Research Technician

Bjarki Johannesson, PhD Student

Markus Mall, MD
Group Leader
now: Head of the Department of Paediatrics, Division of Pneumology and Immunology at Charité University Hospital Berlin

Gregor Reither, PhD, Postdoc

Verena Rickert-Zacharias, PhD student (EMBL International PhD Programme - EIPP)

Susanne Schubert, PhD, Postdoc

Carsten Schultz, PhD
Group Leader
now:Chair of Physiology and Pharmacology at the Portland School of Medicine at OHSU (Oregon Health & State University)

Stephan Spahn, MD student (HBIGS, JCF HRCMM)

Frank Stein, PhD, Postdoc

Joanna Trojanek, PhD, Postdoc

Claudius Wagner,PhD student (HBIGS)



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