Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor

Am J Respir Crit Care Med. 2015 Nov 15;192(10):1252-5. doi: 10.1164/rccm.201507-1271LE.

Authors

Simon Y Graeber^{1

2}, Martin J Hug³, Olaf Sommerburg¹, Stephanie Hirtz^{1

2}, Julia Hentschel⁴, Andrea Heinzmann⁵, Christian Dopfer⁶, Angela Schulz^{6

2}, Jochen G Mainz⁴, Burkhard Tümmler^{6

2}, Marcus A Mall^{1

2}

Affiliations

¹ 1 University of Heidelberg Heidelberg, Germany.
² 6 German Center for Lung Research (DZL).
³ 2 University Medical Center Freiburg Freiburg, Germany.
⁴ 3 Jena University Hospital Jena, Germany.
⁵ 4 University of Freiburg Freiburg, Germany.
⁶ 5 Hannover Medical School Hannover, Germany.

PMID: 26568242
DOI: 10.1164/rccm.201507-1271LE

No abstract available

Publication types

Letter
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Aminophenols / therapeutic use*
Cystic Fibrosis / drug therapy*
Cystic Fibrosis / genetics*
Cystic Fibrosis Transmembrane Conductance Regulator / drug effects*
Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
Female
Humans
Male
Mutation / drug effects
Mutation / genetics
Precision Medicine / methods
Quinolones / therapeutic use*

Substances

Aminophenols
CFTR protein, human
Quinolones
Cystic Fibrosis Transmembrane Conductance Regulator
ivacaftor