Carla Maria Pedrosa Ribeiro, PhD
Professor of Medicine
Joint Professor of Cell Biology and Physiology
Areas of Interest
Cystic Fibrosis, Asthma, COPD, non-CF bronchiectasis, airway inflammation, airway epithelia, endoplasmic reticulum stress, unfolded protein response, mucins
My laboratory studies mechanisms of inflammatory responses relevant to airway diseases characterized by inflammation and mucin overproduction, e.g., cystic fibrosis (CF), asthma, and chronic obstructive pulmonary disease. In particular, we study the role of the Unfolded Protein Response (UPR) in these airway diseases. We were the first to implicate the UPR pathway mediated by the ubiquitous inositol requiring enzyme 1α (IRE1α) in cytokine production by human bronchial epithelia, using models relevant to CF. We also made the key discovery that the isoform IRE1β is only expressed in mucous cells, and required for allergic inflammation-induced airway epithelial mucin overproduction. Recent findings implicating IRE1β in the pathogenesis of idiopathic pulmonary fibrosis associated with airway epithelial mucin overproduction expanded the importance of IRE1β in lung diseases characterized by increased mucus production and airway obstruction. My research has also been directed to studying the impact of CF airway epithelial inflammation on the efficacy of CFTR modulators currently used in the CF clinic. Utilizing pre-clinical models relevant to inflamed CF airways from pediatric and adult CF subjects, we found that airway epithelial inflammation augments the rescue of mutant CFTR by current CFTR modulator therapies. Our long-term goal is to address the mechanism of inflammation-enhanced CFTR rescue, and the interplay between levels of airway epithelial inflammation, anti-inflammatory therapies, and efficacy of CFTR modulators.
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