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Primary Nasal Cell Culture as a Tool for Personalized Therapy in Cystic Fibrosis — Epiithelix  

Cystic Fibrosis Clinical Trial

Official title:
Evaluation of the Primary Human Nasal Epithelial Cell Culture Model in the Context of Personalized Therapy in Cystic Fibrosis

Clinical Trial Summary

characterization of CFTR function and expression in nasal primary cells collected from patients with cystic fibrosis in comparison to their parents, healthy heterozygotes and healthy controls

Clinical Trial Description

3 groups of subjects are enrolled CF subjects according to their genotypes (aiming to enroll patients carrying 2 CF causing mutations with no CFTR expression/function, and patients carrying at least 1 mutation with residual function, such R117H) Parents or siblings of the CF subjects, as healthy hétérozygotes healthy controls All these subjects experience nasal brushings. From these nasal brushings,nasal cells are expanded, and cultured in air liquid interface to obtain polarized epithelium. This epithelium is then studied in Ussing chamber experiments to characterize the level of cAMP dependant Chloride transport and Sodium reabsorption. Apical expression of CFTR is assessed by immunofluorescence.

Results will allow to define the variability of CFTR function and expression criteria in subjects with the same genotype. Such data are crucial for interpretation of the effect of CFTR modulators. ;

Study Design

Related Conditions & MeSH terms

Clinical Trial Details
NCT number NCT03652090
Study type Observational
Source Institut National de la Santé Et de la Recherche Médicale, France
Contact
Status Completed
Phase
Start date September 1, 2010
Completion date March 3, 2016
View Details
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