CareLit · DOI-Nachweis · Pneumologie
ICM is sensitive to detect potentiation of CFTR-mediated Cl- secretion in patients with cystic fibrosis and the G551D mutation treated with ivacaftor
DOI 10.1055/s-0034-1376786
Bibliografische Angaben
Nachweis in CareLit
Autor:innenSY Graeber, MJ Hug, O Sommerburg, JG Mainz, J Hentschel, A Heinzmann, B Tuemmler, MA Mall
ZeitschriftPneumologie
Ausgabe06/2014
Jahrgang / SeitenNicht angegeben
Kernaussage aus der Literatur
Abstract-Vorschau
Background: Sensitive outcome measures of CFTR function may facilitate the implementation of mutation-specific therapy with CFTR modulators in patients with cystic fibrosis with non-G551D mutations. Intestinal current measurement (ICM) is a sensitive assay for functional assessment of mutant CFTR in rectal biopsies and was recently shown to detect potentiator effects of 1-EBIO ex vivo (Roth E. et al., PLOS ONE 2011)…
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Zitierempfehlung
SY Graeber, MJ Hug, O Sommerburg, JG Mainz, J Hentschel, A Heinzmann, B Tuemmler, MA Mall (2014) ICM is sensitive to detect potentiation of CFTR-mediated Cl- secretion in patients with cystic fibrosis and the G551D mutation treated with ivacaftor. Pneumologie (06). https://doi.org/10.1055/s-0034-1376786
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