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Article

Intrinsic Abnormalities of Cystic Fibrosis Airway Connective Tissue Revealed by an In Vitro 3D Stromal Model

1
Istituto Italiano di Tecnologia (IIT), Largo Barsanti e Matteucci 53, 80125 Napoli, Italy
2
Telethon Institute of Genetics and Medicine (TIGEM), Via Campi Flegrei 34, 80078 Pozzuoli (NA), Italy
3
Department of Chemical, Materials and Industrial Production Engineering (DICMAPI) University of Naples Federico II, P. le Tecchio 80, 80125 Napoli, Italy
4
Interdisciplinary Research Centre on Biomaterials (CRIB), University of Napoli Federico II, P. le Tecchio 80, 80125 Napoli, Italy
*
Authors to whom correspondence should be addressed.
Co-last authors.
Cells 2020, 9(6), 1371; https://doi.org/10.3390/cells9061371
Received: 10 April 2020 / Revised: 20 May 2020 / Accepted: 28 May 2020 / Published: 1 June 2020
Cystic fibrosis is characterized by lung dysfunction involving mucus hypersecretion, bacterial infections, and inflammatory response. Inflammation triggers pro-fibrotic signals that compromise lung structure and function. At present, several in vitro cystic fibrosis models have been developed to study epithelial dysfunction but none of these focuses on stromal alterations. Here we show a new cystic fibrosis 3D stromal lung model made up of primary fibroblasts embedded in their own extracellular matrix and investigate its morphological and transcriptomic features. Cystic fibrosis fibroblasts showed a high proliferation rate and produced an abundant and chaotic matrix with increased protein content and elastic modulus. More interesting, they had enhanced pro-fibrotic markers and genes involved in epithelial function and inflammatory response. In conclusion, our study reveals that cystic fibrosis fibroblasts maintain in vitro an activated pro-fibrotic state. This abnormality may play in vivo a role in the modulation of epithelial and inflammatory cell behavior and lung remodeling. We argue that the proposed bioengineered model may provide new insights on epithelial/stromal/inflammatory cells crosstalk in cystic fibrosis, paving the way for novel therapeutic strategies. View Full-Text
Keywords: cystic fibrosis; connective airway tissue; lung fibroblasts; extracellular matrix; 3D bioengineered model; RNA sequencing cystic fibrosis; connective airway tissue; lung fibroblasts; extracellular matrix; 3D bioengineered model; RNA sequencing
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MDPI and ACS Style

Mazio, C.; Scognamiglio, L.S.; De Cegli, R.; Galietta, L.J.V.; Di Bernardo, D.; Casale, C.; Urciuolo, F.; Imparato, G.; Netti, P.A. Intrinsic Abnormalities of Cystic Fibrosis Airway Connective Tissue Revealed by an In Vitro 3D Stromal Model. Cells 2020, 9, 1371. https://doi.org/10.3390/cells9061371

AMA Style

Mazio C, Scognamiglio LS, De Cegli R, Galietta LJV, Di Bernardo D, Casale C, Urciuolo F, Imparato G, Netti PA. Intrinsic Abnormalities of Cystic Fibrosis Airway Connective Tissue Revealed by an In Vitro 3D Stromal Model. Cells. 2020; 9(6):1371. https://doi.org/10.3390/cells9061371

Chicago/Turabian Style

Mazio, Claudia, Laura S. Scognamiglio, Rossella De Cegli, Luis J.V. Galietta, Diego Di Bernardo, Costantino Casale, Francesco Urciuolo, Giorgia Imparato, and Paolo A. Netti. 2020. "Intrinsic Abnormalities of Cystic Fibrosis Airway Connective Tissue Revealed by an In Vitro 3D Stromal Model" Cells 9, no. 6: 1371. https://doi.org/10.3390/cells9061371

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