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Mechanisms and clinical implications of endothelial dysfunction in cystic fibrosis
     
 
Author(s) : Totani L, Plebani R, Patruno S, Piccoli A, Di Silvestre S, Lanuti P, Recchiuti A, Cianci E, Dell’Elba G, Sacchetti S, Guarnieri S, Mariggiò MA, Mari VC, Anile M, Venuta F, Del Porto P, Moretti P, Prioletta M, Mucilli F, Marchisio M, Pandolfi A, Evangelista V, Romano M
Address : G. D’Annunzio University, Chieti-Pescara, Italy; Italian Institute of Technology, Genoa, Italy; University of Rome “Sapienza” Rome, Italy;"Charles Darwin" Sapienza University, Rome, Italy;S. Liberatore Hospital, Italy.
Multiple Universities in collaboration
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Background. Despite the involvement of endothelial cells (EC) in cystic fibrosis (CF) pathogenesis is suggested by (1)the emerging evidence of the risk for cardiovascular events in CF patients as well as by (2) our work showing endothelial dysfunction and its correlation with lung disease in CF patients, EC have not been extensively studied in CF. 

Hypothesis and objectives. We hypothesized that the elucidation of CFTR signaling in EC may provide clues for innovative pharmacology for CF. Main objectives of this study were to: 1. Uncover mechanisms of CF endothelial dysfunction as molecular targets for novel therapeutics. 2. Elucidate the clinical relevance of circulating endothelial cells (CEC) and endothelial microvesicles (EMV) in CF.

 
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