A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor

To determine if oral dosing with the CFTR-potentiator ivacaftor (VX-770, Kalydeco) improves CFTR-dependent sweating in CF subjects carrying G551D or R117H-5T mutations, we optically measured sweat secretion from 32-143 individually identified glands in each of 8 CF subjects; 6 F508del/G551D, one G55...

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Veröffentlicht in:	PloS one 2014-02, Vol.9 (2), p.e88564
Hauptverfasser:	Char, Jessica E, Wolfe, Marlene H, Cho, Hyung-Ju, Park, Il-Ho, Jeong, Jin Hyeok, Frisbee, Eric, Dunn, Colleen, Davies, Zoe, Milla, Carlos, Moss, Richard B, Thomas, Ewart A C, Wine, Jeffrey J
Format:	Artikel
Sprache:	eng
Schlagworte:	Acetylcholine receptors (muscarinic) Amino Acid Substitution - genetics Aminophenols - pharmacology Aminophenols - therapeutic use Analysis Bioassays Biology Case-Control Studies Chloride Cystic fibrosis Cystic Fibrosis - drug therapy Cystic Fibrosis - genetics Cystic Fibrosis Transmembrane Conductance Regulator - genetics Data processing FDA approval Female Glands Humans Laboratories Male Medicine Mutation Pediatrics Quinolones - pharmacology Quinolones - therapeutic use Receptors Secretion Studies Suidae Sweat Sweat - drug effects Sweat - metabolism Sweat Glands - drug effects Sweat Glands - metabolism Sweat Glands - pathology Sweating Transcription
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