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Epigenetic modifiers has been showed to rescue F508del-CFTR channel to apical membrane of epithelial cell lines. In this study, the investigators evaluate epigenetic modifiers effects firstly on CFTR rescue, then on secretion and synthesis of inflammatory factors (IL-8, LXA4 and SCGB1A1) and mucines (MUC5AC and MUC5B) in a dynamic epithelium model using an air-liquide interface culture of nasals cells from CF patients or controls.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Cystic Fibrosis patients (CF) | Experimental | Tests in vitro after sampling nasal cells of CF patients or controls are the intervention done on these subjects |
|
| Control subjects (non CF) | Other | Tests in vitro after sampling nasal cells of CF patients or controls are the intervention done on these subjects |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Tests in vitro after sampling nasal cells of CF patients or controls | Other | Nasal cells of CF patients or controls were collected by scratching of intermediate turbinate and cultured in vitro. Epigenetic modifiers treatment was applied to these ex vivo nasal epithelia. Then, mRNA, protein and secretions were quantified. |
| Measure | Description | Time Frame |
|---|---|---|
| Mature CFTR protein percentage variation after in vitro epigenetics modifiers treatment | 24 months |
| Measure | Description | Time Frame |
|---|---|---|
| Pro-inflammatory cytokines number variations after epigenetic modifiers treatment | 24 months | |
| Anti-inflammatory cytokines number variations after epigenetic modifiers treatment | 24 months | |
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General inclusion criteria :
Inclusion Criteria for CF patients
Inclusion Criteria for controls :
General exclusion criteria :
Specific Exclusion Criteria:
Specific Control subject Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Raphaƫl RC CHIRON, MD | Arnaud de Villeneuve CHU Montpellier | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Respiratory Diseases Department | Montpellier | 34295 | France |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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|
| Mucin composition variations after epigenetic modifiers treatment |
| 24 months |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |