CFTR and Ca2+ Signaling in Cystic Fibrosis - Archive ouverte HAL
Article Dans Une Revue Frontiers in Pharmacology Année : 2011

CFTR and Ca2+ Signaling in Cystic Fibrosis

Résumé

Among the diverse physiological functions exerted by calcium signaling in living cells, its role in the regulation of protein biogenesis and trafficking remains incompletely understood. In cystic fibrosis (CF) disease the most common CF transmembrane conductance regulator (CFTR) mutation, F508del-CFTR generates a misprocessed protein that is abnormally retained in the endoplasmic reticulum (ER) compartment, rapidly degraded by the ubiquitin/proteasome pathway and hence absent at the plasma membrane of CF epithelial cells. Recent studies have demonstrated that intracellular calcium signals consequent to activation of apical G-protein-coupled receptors by different agonists are increased in CF airway epithelia. Moreover, the regulation of various intracellular calcium storage compartments, such as ER is also abnormal in CF cells. Although the molecular mechanism at the origin of this increase remains puzzling in epithelial cells, the F508del-CFTR mutation is proposed to be the onset of abnormal Ca(2+) influx linking the calcium signaling to CFTR pathobiology. This article reviews the relationships between CFTR and calcium signaling in the context of the genetic disease CF.
Fichier principal
Vignette du fichier
CFTR and Ca2+ signaling in cystic fibrosis.pdf (867.3 Ko) Télécharger le fichier
Origine Fichiers éditeurs autorisés sur une archive ouverte

Dates et versions

hal-00664651 , version 1 (25-06-2024)

Identifiants

Citer

Fabrice Antigny, Caroline Norez, Frédéric Becq, Clarisse Vandebrouck. CFTR and Ca2+ Signaling in Cystic Fibrosis. Frontiers in Pharmacology, 2011, 2, pp.67. ⟨10.3389/fphar.2011.00067⟩. ⟨hal-00664651⟩
117 Consultations
12 Téléchargements

Altmetric

Partager

More