Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.
Journal
Scientific reports
ISSN: 2045-2322
Titre abrégé: Sci Rep
Pays: England
ID NLM: 101563288
Informations de publication
Date de publication:
12 04 2022
12 04 2022
Historique:
received:
01
08
2021
accepted:
28
02
2022
entrez:
13
4
2022
pubmed:
14
4
2022
medline:
15
4
2022
Statut:
epublish
Résumé
Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) are responsible for Cystic Fibrosis (CF). The most common CF-causing mutation is the deletion of the 508th amino-acid of CFTR (F508del), leading to dysregulation of the epithelial fluid transport in the airway's epithelium and the production of a thickened mucus favoring chronic bacterial colonization, sustained inflammation and ultimately respiratory failure. c407 is a bis-phosphinic acid derivative which corrects CFTR dysfunction in epithelial cells carrying the F508del mutation. This study aimed to investigate c407 in vivo activity in the F508del Cftr
Identifiants
pubmed: 35413967
doi: 10.1038/s41598-022-09678-9
pii: 10.1038/s41598-022-09678-9
pmc: PMC9005718
doi:
Substances chimiques
Chlorides
0
Phosphinic Acids
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
6132Informations de copyright
© 2022. The Author(s).
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