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Am J Physiol Lung Cell Mol Physiol. 2019 Sep 11. doi: 10.1152/ajplung.00090.2019. [Epub ahead of print]

Characterization of Cystic Fibrosis Airway Smooth Muscle Cell Proliferative and Contractile Activities.

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Meakins-Christie Laboratories, McGill University, Canada.
Department of Medicine, Meakins-Christie Laboratories, McGill University, Canada.
Department of Medicine, Research Institute of the McGill University Health Centre, Canada.
Meakins-Christie Laboratories RI-MUHC- McGill University 1001 Decarie Blvd, McGill University, Canada.


Cystic Fibrosis (CF) is a genetic disease that causes multiple pathologies in the airway. Two major respiratory symptoms of CF are airway hyperresponsiveness (AHR) and airway remodeling. Airway smooth muscle (ASM) is hypothesized to be responsible for these airway dysfunctions as their thickening is involved in remodeling and excessive contraction by the ASM may be behind AHR. It is unclear whether the ASM are intrinsically pathologic, or if micro-environmental influences induce pathological behavior in the ASM. In this study, the contractile and proliferative properties of ASM cells isolated from healthy donor and CF transplant lungs were compared. Proliferation assays showed CF ASM proliferate at a higher rate than healthy cells. Through calcium analysis, no differences in contractile activation was found, however, CF ASM lagged in their reuptake of calcium. The combination CFTR corrector and potentiator, VX-809/770, was used to restore proper CFTR function in CF ASM which resulted in a reduction in proliferation and in a normalization of calcium reuptake kinetics. These results show that improper CFTR function in ASM cause intrinsic changes in their proliferative and contractile properties.


CFTR; Hyperresponsiveness; Remodeling; Vertex; calcium


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