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Professor of Medicine

Professor of Medicine, Division of Pulmonary Diseases and Critical Care Medicine
Joint Associate Professor of Cell Biology and Physiology
Marsico Lung Institute
Cystic Fibrosis Center

Specialty Areas: Mechanisms of Airway Inflammation in CF, COPD, and Asthma

Chronology: PhD: Duke University, 1992; Postdoctoral: NIH/NIEHS, 1993-1998; Research Associate, University of North Carolina, 1998-2001; Assistant Professor of Medicine, University of North Carolina, 2001-2010; Associate Professor of Medicine, University of North Carolina, 2010-present; Joint Associate Professor of Cell Biology and Physiology, University of North Carolina, July 2011-present.

Dr. Ribeiro is a pulmonary biologist who has developed translational models to study airway inflammatory diseases. Research in the Ribeiro laboratory focuses on studying mechanisms of airway inflammatory responses relevant to the pathogenesis of airway diseases characterized by mucus obstruction, inflammation, and oxidative stress, such as cystic fibrosis (CF), chronic obstructive pulmonary disease (COPD) and asthma. In particular, the Ribeiro laboratory studies the functional roles of the endoplasmic reticulum (ER) and the mitochondria in the regulation of intracellular calcium (Ca2+i) signals and Ca2+i-mediated inflammation, and ER stress responses pertinent to the pathophysiology of these pulmonary diseases.

The Ribeiro group was the first to implicate the ER stress 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. The 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.

Dr. Ribeiro has received funding from several agencies, including the NIH (NHLBI, NIAID), the Cystic Fibrosis Foundation, and the American Asthma Foundation. She has served as a reviewer of grant applications for multiple NIH Study sections, the Cystic Fibrosis Foundation, the European Science Foundation, the Irish Health Research Board, the Research Grants Council (Hong Kong), and the Research Foundation – Flanders (Fonds Wetenschappelijk Onderzoek – Vlaanderen, FWO; Belgium). In addition, Dr. Ribeiro has reviewed manuscripts for several Journals, including the American Journal of Respiratory and Critical Care Medicine, the American Journal of Respiratory Cell and Molecular Biology, the European Respiratory Journal, the Journal of Cystic Fibrosis, Mucosal Immunology, The American Journal of Physiology – Lung Cellular and Molecular Physiology, and The Journal of Clinical Investigation. Dr. Ribeiro has recently served as Editor for a Thematic Issue in Frontiers in Pharmacology, “Novel Anti-inflammatory Approaches for Cystic Fibrosis Lung Disease: Identification of Molecular Targets and Design of Innovative Therapies”, and is serving as a Guest Associate Editor for Frontiers Inflammation Pharmacology. Dr. Ribeiro has mentored numerous persons in her lab and serves/has served in several committees to foster the teaching mission of the UNC School of Medicine.

Dr. Ribeiro was one of three investigators honored by the UNC Eshelman School of Pharmacy as an innovator for work that helped lay the foundation to develop a new class of therapeutics for respiratory diseases. Read the full story here.

Selected Publications:

Refereed Articles: Original Research

Gentzsch, M., Cholon, D. M., Quinney, N. L., Martino, M. E. B., Minges, J. T., Boyles, S. E., Lee, T. N. G., Esther, C. R., and Ribeiro, C. M. P. (2021): Airway epithelial inflammation in vitro augments the rescue of mutant CFTR by current CFTR modulator therapies (in press, Frontiers in Pharmacology).

Hull-Ryde, E. A.; Minges, J. T.; Martino, M. E. B.; Kato, T.; Norris-Drouin, J.L.; Ribeiro, C. M. P. (2021): IRE1a Is a Therapeutic Target for Cystic Fibrosis Airway Inflammation. Int. J. Mol. Sci., 22, 3063. https://doi.org/10.3390/ijms22063063

Chen, G., Ribeiro, C. M. P., Sun, L., Okuda, K., Kato, T., Gilmore, R. C., Martino, M. B., Dang, H., Abzhanova, A., Lin, J. M., Hull-Ryde, E. A., Volmer, A. S., Randell, S. H., Livraghi-Butrico, A., Deng, Y., Scherer, P. E., Stripp, B. R., O’Neal, W. K., Boucher, R. C. (2019): XBP1S Regulates MUC5B in a Promoter Variant-Dependent Pathway in IPF Airway Epithelia. Am J Respir Crit Care Med., 2019 Jul 15;200:220-234. doi: 10.1164/rccm.201810-1972OC.

Related editorial: https://www.atsjournals.org/doi/pdf/10.1164/rccm.201904-0809ED

Gentzsch, M., Cholon, D. M., Quinney, N. L., Boyles, S. E., Martino, M. E. B., and Ribeiro, C. M. P. (2018): The Cystic Fibrosis Airway Milieu Enhances Rescue of F508del in a Pre-Clinical Model. Eur Respir J. 2018 Dec 20;52(6). pii: 1801133. doi: 10.1183/13993003.01133-2018. Print 2018 Dec.

Press release: http://news.unchealthcare.org/news/2018/october/scientists-reveal-new-cystic-fibrosis-treatments-work-best-in-inflamed-airways?utm_source=vs-email&utm_medium=email&utm_campaign=41

Abdullah, L. H., Coakley, R., Zhu, Y., Webster, M. J., Tarran, R., Radicioni, G., Kesimer, M., Boucher, R. C., Davis, C. W., and Ribeiro, C. M. P. (2018): Mucin Production and Hydration Responses to Mucopurulent Materials in Normal vs. CF Airway Epithelia. Am. J. Respir. Crit. Care Med., 2018 Feb 15;197:481-491.

Related Editorial: https://www.atsjournals.org/doi/abs/10.1164/rccm.201712-2554ED?url_ver=Z39.882003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub%3Dpubmed#readcube-epdf

Press release: http://news.unchealthcare.org/news/2017/november/a-new-test-to-measure-the-effectiveness-of-cf-drugs.

Lubamba, B. A., Jones, L. C., O’Neal, W. K., Boucher, R. C. and Ribeiro, C. M. P. (2015): X-Box Binding Protein 1 and Innate Immune Responses of Human Cystic Fibrosis Alveolar Macrophages. Am J Respir Crit Care Med. 2015 Dec 15;192(12):1449-61.

Related Editorial: http://www.atsjournals.org/doi/abs/10.1164/rccm.201509-1811ED

Press release: https://healthtalk.unchealthcare.org/researchers-further-illuminate-pathway-for-treatment-of-cystic-fibrosis/

Martino, M. E. B., Jones, L., Brighton, B., Ehre, C., Abdulah, L., Davis, C. W., Ron, D., O’Neal, W. K. and Ribeiro, C. M. P. (2013): The ER Stress Transducer IRE1β is Required for Airway Epithelial Mucin Production. Mucosal Immunol. 2013 May;6(3):639–654. “Featured Article” in the May 2013 Mucosal Immunology issue.

Ribeiro, C. M. P., Hurd, H., Wu, Y., Martino, M. E. B., Jones, L., Brighton, B., Boucher, R. C., and O’Neal, W. K. (2009): Azithromycin Treatment Alters Gene Expression in Inflammatory, Lipid Metabolism, and Cell Cycle Pathways in Well-Differentiated Human Airway Epithelia. PLoS One. 2009 Jun 5;4(6):e5806.

Martino, M. E. B., Olsen, J. C., Fulcher, N. B., Wolfgang, M. C., O’Neal, W. K., and Ribeiro, C. M. P. (2009): Airway epithelial inflammation-induced endoplasmic reticulum Ca2+ store expansion is mediated by X-box binding protein-1. J. Biol. Chem. 2009 May 29;284(22):14904-13.

Livraghi A., Mall M., Paradiso A.M., Boucher R.C., and Ribeiro, C.M. P. (2008): Modeling dysregulated Na+ absorption in airway epithelial cells with mucosal nystatin treatment. Am. J. Respir. Cell Mol. Biol. 2008 Apr;38(4):423-34.

Ribeiro C. M. P., Paradiso, A. M., Schwab, U., Perez-Vilar, J., Jones, L., O’Neal, W., and Boucher, R. C. (2005): Chronic Airway Infection/Inflammation Induces a Ca2+i-dependent Hyperinflammatory Response in Human Cystic Fibrosis Airway Epithelia. J Biol Chem. 2005 May 6;280(18):17798-17806.

Ribeiro, C. M. P., Paradiso, A. M., Carew, M. A., Shears, S. B., and Boucher, R. C. (2005): Cystic fibrosis airway epithelial Ca2+i signaling. The mechanism for the larger agonist-mediated Ca2+i signals in human cystic fibrosis airway epithelia. J. Biol.Chem. 2005 Mar 18;280(11):10202-10209.

Ribeiro, C. M. P., Paradiso, A. M., Livraghi, A., and Boucher, R. C. (2003): The mitochondrial barriers segregate agonist-induced calcium-dependent functions in human airway epithelia. J. Gen. Physiol. 2003 Oct;122(4): 377-387.

Paradiso, A. M., Ribeiro, C. M., and Boucher, R. C. (2001): Polarized Signaling via Purinoceptors in Normal and Cystic Fibrosis Airway Epithelia. J. Gen. Physiol. 2001 Jan;117(1): 53-67.

Refereed Articles: Reviews

Ribeiro, C. M. P., Lubamba, B. A. (2017): Role of IRE1α/XBP-1 in Cystic Fibrosis Airway Inflammation. Int J Mol Sci. 2017 Jan 9;18(1). pii: E118. doi: 10.3390/ijms18010118.

Ribeiro, C. M. P. and O’Neal, W. K. (2012): ER Stress in Chronic Obstructive Lung Diseases. Curr Mol Med. 12:872-82..

Ribeiro C. M. P., Boucher RC. (2010): Role of endoplasmic reticulum stress in cystic fibrosis-related airway inflammatory responses. Proc. Am. Thorac. Soc. 7:387-94.

Ribeiro, C. M. P. (2006): The Role of Intracellular Calcium Signals in Inflammatory Responses of Polarised Cystic Fibrosis Human Airway Epithelia. Drugs in R & D. 7:17-31.

 

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