Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.

doi:10.1016/j.jcf.2016.09.003

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Article

Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Nov 2016

Sermet-Gaudelus Isabelle, Delion Martial, Durieu Isabelle, Jacquot Jacky, Hubert Dominique

Abstract excerpt

Low bone mineral density (BMD) is a common problem in adults with cystic fibrosis (CF), the etiology of which is multifactorial. In this study, we provide the first evidence that ivacaftor improves BMD in CF patients carrying the p.Gly551Asp mutation. Consistently, in vitro experiments with TNF-α-stimulated primary F508del-CFTR osteoblasts demonstrated that correction of p.Phe508del-CFTR markedly decreased RANKL...

Topics

Adult
Aminophenols
Bone Demineralization, Pathologic
Bone Density
Cells, Cultured
Chloride Channel Agonists
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Female
Humans

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Male

Middle Aged

Mutation

Osteoblasts

Quinolones

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Identifiers

PMID: 27745802
DOI: 10.1016/j.jcf.2016.09.003

Genes

CFTR
TNF

Variants

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