Pubblicazioni

Autori:

Pedrazzi, Marco; Vercellone, Silvia; Barberis, Elettra; Capraro, Michela; De Tullio, Roberta; Cresta, Federico; Casciaro, Rosaria; Castellani, Carlo; Patrone, Mauro; Marengo, Emilio; Lecca, Paola; Melotti, Paola; Sorio, Claudio; Manfredi, Marcello; Averna, Monica

Titolo:

Identification of Potential Leukocyte Biomarkers Related to Drug Recovery of CFTR: Clinical Applications in Cystic Fibrosis

Anno:

2021

Tipologia prodotto:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Lingua:

Inglese

Formato:

Elettronico

Referee:

No

Nome rivista:

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

ISSN Rivista:

1422-0067

N° Volume:

22

Numero o Fascicolo:

8

Intervallo pagine:

3928-3943

Parole chiave:

Ivacaftor; MMP9; PBMCs; VX770; monocytes; proteomics

Breve descrizione dei contenuti:

The aim of this study was the identification of specific proteomic profiles, related to a restored cystic fibrosis transmembrane conductance regulator (CFTR) activity in cystic fibrosis (CF) leukocytes before and after ex vivo treatment with the potentiator VX770. We used leukocytes, isolated from CF patients carrying residual function mutations and eligible for Ivacaftor therapy, and performed CFTR activity together with proteomic analyses through micro-LC-MS. Bioinformatic analyses of the results obtained revealed the downregulation of proteins belonging to the leukocyte transendothelial migration and regulation of actin cytoskeleton pathways when CFTR activity was rescued by VX770 treatment. In particular, we focused our attention on matrix metalloproteinase 9 (MMP9), because the high expression of this protease potentially contributes to parenchyma lung destruction and dysfunction in CF. Thus, the downregulation of MMP9 could represent one of the possible positive effects of VX770 in decreasing the disease progression, and a potential biomarker for the prediction of the efficacy of therapies targeting the defect of Cl- transport in CF.

Id prodotto:

120975

Handle IRIS:

11562/1043042

ultima modifica:

16 maggio 2024

Citazione bibliografica:

Pedrazzi, Marco; Vercellone, Silvia; Barberis, Elettra; Capraro, Michela; De Tullio, Roberta; Cresta, Federico; Casciaro, Rosaria; Castellani, Carlo; Patrone, Mauro; Marengo, Emilio; Lecca, Paola; Melotti, Paola; Sorio, Claudio; Manfredi, Marcello; Averna, Monica, Identification of Potential Leukocyte Biomarkers Related to Drug Recovery of CFTR: Clinical Applications in Cystic Fibrosis «INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES» , vol. 22 , n. 8 , 2021 , pp. 3928-3943

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