Dataset Information
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
Ontology highlight
ABSTRACT:
SUBMITTER: Skilton M
PROVIDER: S-EPMC6353056 | biostudies-literature | 2019 Jan
REPOSITORIES: biostudies-literature
Publications
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
The Cochrane database of systematic reviews 20190107
<h4>Background</h4>Cystic fibrosis (CF) is the commonest inherited life-shortening illness in white populations, caused by a mutation in the gene that codes for the cystic fibrosis transmembrane regulator protein (CFTR), which functions as a salt transporter. This mutation mainly affects the airways where excess salt absorption dehydrates the airway lining leading to impaired mucociliary clearance. Consequently, thick, sticky mucus accumulates making the airway prone to chronic infection and pro ...[more]