Intestinal current measurements detect activation of mutant CFTR in patients with cystic fibrosis with the G551D mutation treated with ivacaftor

American Journal of Respiratory and Critical Care Medicine

Volumn 192, Issue 10, 2015, Pages 1252-1255

  Graeber, Simon Y ^a,b   Hug, Martin J ^c   Sommerburg, Olaf ^a   Hirtz, Stephanie ^a,b   Hentschel, Julia ^d   Heinzmann, Andrea ^e   Dopfer, Christian ^f   Schulz, Angela ^b,f   Mainz, Jochen G ^d   Tümmler, Burkhard ^b,f   Mall, Marcus A ^a,b  

^a UNIVERSITY OF HEIDELBERG   (Germany)

^b German Center for Lung Research (DZL)   (United States)

^c UNIVERSITY OF FREIBURG   (Germany)

^d JENA UNIVERSITY HOSPITAL   (Germany)

^e UNIVERSITY OF FREIBURG   (Germany)

^f HANNOVER MEDICAL SCHOOL   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

AMILORIDE; CARBACHOL; CHLORIDE CHANNEL; CYCLIC AMP DEPENDENT PROTEIN KINASE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; FORSKOLIN; INDOMETACIN; ISOBUTYLMETHYLXANTHINE; IVACAFTOR; SODIUM; AMINOPHENOL DERIVATIVE; CFTR PROTEIN, HUMAN; QUINOLONE DERIVATIVE;

ADOLESCENT; ADULT; BODY MASS; CLINICAL ARTICLE; COMPARATIVE STUDY; CONTROLLED STUDY; CYSTIC FIBROSIS; FEMALE; FORCED EXPIRATORY VOLUME; GASTROENTEROLOGICAL PROCEDURE; GENE MUTATION; GENOTYPE; HUMAN; HUMAN TISSUE; INTESTINAL CURRENT MEASUREMENT; LETTER; LUNG FUNCTION; MALE; MEASUREMENT; MEASUREMENT ACCURACY; OBSERVATIONAL STUDY; OUTCOME ASSESSMENT; PRIORITY JOURNAL; RECTUM BIOPSY; RECTUM MUCOSA; SHORT CIRCUIT CURRENT; TREATMENT RESPONSE; DRUG EFFECTS; GENETICS; MUTATION; PERSONALIZED MEDICINE; PROCEDURES;

ADOLESCENT; AMINOPHENOLS; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; FEMALE; HUMANS; MALE; MUTATION; PRECISION MEDICINE; QUINOLONES;

EID: 84949035982     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201507-1271LE     Document Type: Letter

References (13)

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