Cystic Fibrosis; Lung; Macrophages; Pediatrics; Respiration Disorders; Cystic Fibrosis Transmembrane Conductance Regulator
CPIRT - Pulmonary Infection Research and Treatment
Our research aims to understand how CFTR--the gene that, when mutated, causes Cystic Fibrosis (CF)--affects innate immunity and how this will impact the progression of CF lung disease. We have performed pioneering studies demonstrating that CFTR expression is necessary for the normal function of an important player of the innate immune response: macrophages. Using in vitro and in vivo models, we are dissecting the molecular mechanisms by which CFTR affects these cells.
Specialized Terms: Cystic Fibrosis; Lung inflammation; Macrophages
Extensive Research Description
- Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation. Zhang PX, Cheng J, Zou S, D'Souza AD, Koff JL, Lu J, Lee PJ,n Krause DS, Egan ME, Bruscia EM. Nat Commun. 2015 Feb 10;6:6221.
- Reduced caveolin-1 promotes hyperinflammation due to abnormal heme oxygenase-1 localization in lipopolysaccharide-challenged macrophages with dysfunctional cystic fibrosis transmembrane conductance regulator. Zhang PX, Murray TS, Villella VR, Ferrari E, E
- Innate immunity in cystic fibrosis lung disease (Review). Hartl D, Gaggar A, Bruscia EM, Hector A, Marcos V, Jung A, Greene C, McElvaney G, Mall M, Döring G. J Cyst Fibros. 2012 Sep;11(5):363-82.
- The Carbon Monoxide Releasing Molecule CORM-2 attenuates Pseudomonas aeruginosa biofilm formation. Murray TS, Okegbe C, Gao Y, Kazmierczak BI, Motterlini R, Dietrich LE, Bruscia EM. PLoS One. 2012;7(4):e35499.
- Abnormal trafficking and degradation of TLR4 underlie the elevated inflammatory response in cystic fibrosis. Bruscia EM, Zhang PX, Satoh A, Caputo C, Medzhitov R, Shenoy A, Egan ME, Krause DS. J Immunol. 2011 Jun 15;186(12):6990-8.
- Macrophages directly contribute to the exaggerated inflammatory response in cystic fibrosis transmembrane conductance regulator-/- mice. Bruscia EM, Zhang PX, Ferreira E, Caputo C, Emerson JW, Tuck D, Krause DS, Egan ME. Am J Respir Cell Mol Biol. 2009 Ma
- Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation. Bruscia EM, Price JE, Cheng EC, Weiner S, Caputo C, Ferreira EC, Egan ME, Krause DS. Proc Natl Acad Sci U S A. 2006 Feb