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Lung Repair in People with Cystic Fibrosis

A team of Cystic Fibrosis Canada-funded researchers led by Dr. Emmanuelle Brochiero from the CHUM Research Centre (CRCHUM) and University of Montreal received a $238,254 grant from 2012-2016 to study lung injury and repair in people with cystic fibrosis (CF).

People with CF suffer from chronic lung infections and inflammation, which cause progressive lung damage, a leading cause of illness and death in this population. Dr. Brochiero’s team studied the ability of epithelial cells to repair the lung damage caused by infection and inflammation. They also studied possible ways to improve lung repair in people with CF.

Dr. Brochiero’s work highlights the negative impact of CF on the ability of lungs to repair after injury. It also points to possible ways that bacterial infection may inhibit the function of drugs designed to correct the basic defect in CF (i.e., CFTR correctors).

What did they find?

  • Lungs of people with CF have extensive damage. Moreover, injuries in the lung tissue of people with CF tend to heal more slowly than in lung tissue of people without CF. The defective protein channel in CF, the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), seems to be involved, as CFTR inhibition reduces healing rates, while CFTR rescue with correctors improves the speed of repair.
  • High blood sugar levels, which are associated with Cystic Fibrosis-Related Diabetes, a complication that affects approximately 40% of adults with CF, leads to slower lung epithelial repair.
  • Pseudomonas aeruginosa bacteria, common in the lungs of people with CF, secrete products that are also linked with reduced repair rates. This means that lung infections could contribute, along with the basic CFTR defect, to the incapacity of CF lungs to efficiently repair.
  • The ability of CFTR modulators to correct CFTR protein function are impaired in the presence of Pseudomonas aeruginosa bacterial secretions. Dr. Brochiero’s team is currently investigating the identity of bacterial products responsible for this negative effect, and developing strategies to counteract their deleterious impact, enhancing the effectiveness of CFTR modulators.

To access publications on this work, please refer to:

Improvement of defective cystic fibrosis airway epithelial wound repair after CFTR rescue. Eur Respir J. 2012; 40: 1390–1400. [Abstract]

Deleterious impact of hyperglycermia on cystic fibrosis airway ion transport and epithelial repair. J Cyst Fibros. 2016 Jan; 15(1):43-51. [Abstract]

Quorum-sensing inhibition abrogates the deleterious impact of Pseudomonas aeruginosa on airway

epithelial repair. FASEB J. 2016 May 13. [Abstract]

Deleterious impact of Pseudomonas aeruginosa on cystic fibrosis transmembrane conductance regulator function and rescue in airway epithelial cells. Eur Respir J. 2015 Jun; 45(6): 1590-602. [Abstract]

Bio:

Emmanuelle Brochiero is a researcher at CRCHUM and professor at the Université de Montréal (Faculty of Medicine). She is also the Director of the Respiratory Tissue Biobank of CRCHUM and of the cystic fibrosis strategic group of the Respiratory Health Network of the FRQS. Dr. Brochiero’s research interests focus on the role of pulmonary epithelium in the pathophysiology of cystic fibrosis and acute respiratory distress syndrome. More specifically, she has been devoted to understanding the role of ion channels, inflammation and infection in these diseases. Her goal is to develop novel therapeutic strategies favoring lung repair and the functional rescue of CFTR.

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