Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation

Amit Gaggar*, 1, 2, 3, 4, 5, §, Steven M Rowe*, 1, 2, 3, 4, §, Hardision Matthew1, J. Edwin Blalock1, 2, 4
1 Department of Physiology and Biophysics, University of Alabama at Birmingham, Birmingham, AL
2 Department of Medicine, University of Alabama at Birmingham, Birmingham, AL
3 Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL
4 Gregory Fleming James Cystic Fibrosis Center, University of Alabama at Birmingham, Birmingham, AL
5 Birmingham VA Medical Center, Birmingham, AL, USA

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© Gaggar et al.; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to these authors at the 1900 University Blvd, THT 422 Birmingham, AL 35294, USA; Tel: 205-934-5400; Fax: 205-934-1721; E-mail:,
§ These authors have contributed equally to this work.


Cystic fibrosis (CF) is chronic lung disease characterized by an unrelenting neutrophil-predominant airway inflammatory response. This inflammation leads to extracellular matrix (ECM) remodeling and eventually to the development of bronchiectasis. While many components of the immune response in CF have been well-characterized, recent data suggests that small molecules may play an important and underappreciated role in this inflammation. This review will examine two novel molecules: proline-glycine-proline (PGP) and high mobility group box protein-1 (HMGB1), and their potential impact in CF lung disease. This review will provide a brief overview of CF lung disease and background on both HMGB1 and PGP. It will then focus on these molecules in a murine model of CF-like airway disease and in human biological specimens from CF individuals. Finally, this manuscript will address possible mechanisms for therapeutic targeting of these bioactive mediators.

Keywords: Inflammation, cystic fibrosis, neutrophils, airway, therapeutics..