Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity Pneumonitis
Hypersensitivity pneumonitis (HP) is a diffuse interstitial lung disease (ILD) caused by the inhalation of a variety of antigens in susceptible individuals. Patients with fibrotic HP (fHP) may show histopathological and radiological manifestations similar to patients with idiopathic pulmonary fibros...
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MDPI AG
2021-09-01
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| Series: | Biomedicines |
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| Online Access: | https://www.mdpi.com/2227-9059/9/9/1193 |
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| author | Paolo Giannoni Marco Grosso Giuseppina Fugazza Mario Nizzari Maria Cristina Capra Rita Bianchi Roberto Fiocca Sandra Salvi Fabrizio Montecucco Maria Bertolotto Franco Fais Mario Salio Emanuela Barisione Daniela de Totero |
| author_facet | Paolo Giannoni Marco Grosso Giuseppina Fugazza Mario Nizzari Maria Cristina Capra Rita Bianchi Roberto Fiocca Sandra Salvi Fabrizio Montecucco Maria Bertolotto Franco Fais Mario Salio Emanuela Barisione Daniela de Totero |
| author_sort | Paolo Giannoni |
| collection | DOAJ |
| description | Hypersensitivity pneumonitis (HP) is a diffuse interstitial lung disease (ILD) caused by the inhalation of a variety of antigens in susceptible individuals. Patients with fibrotic HP (fHP) may show histopathological and radiological manifestations similar to patients with idiopathic pulmonary fibrosis (usual interstitial pneumonia-like pattern of fibrosis) that are associated with a worse prognosis. We describe here the establishment and characterization of a fibroblastic cell line derived from the broncho-alveolar lavage (BAL) of a patient with fHP, a 53 year old man who presented at our Pneumology Unit with cough and dyspnea. The fHP diagnosis was based on international criteria and multidisciplinary discussion. Primary fibroblasts were expanded in vitro until passage 36. These fibroblasts displayed morpho/phenotypical features of myofibroblasts, showing high positivity for α-smooth muscle actin, type I collagen, and fibronectin as determined by quantitative RT-PCR and cyto-fluorographic analysis. Cytogenetic analyses further evidenced trisomy of chromosome 10, which interestingly harbors the FGF2R gene. To our knowledge, this is the first fibroblastic cell line derived from an fHP patient and might, therefore, represent a suitable tool to model the disease in vitro. We preliminarily assessed here the activity of pirfenidone, further demonstrating a consistent inhibition of cells growth by this antifibrotic drug. |
| first_indexed | 2024-03-10T07:52:41Z |
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| id | doaj.art-d29167c2d89b4aeb9362d9febf589267 |
| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-10T07:52:41Z |
| publishDate | 2021-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomedicines |
| spelling | doaj.art-d29167c2d89b4aeb9362d9febf5892672023-11-22T12:08:25ZengMDPI AGBiomedicines2227-90592021-09-0199119310.3390/biomedicines9091193Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity PneumonitisPaolo Giannoni0Marco Grosso1Giuseppina Fugazza2Mario Nizzari3Maria Cristina Capra4Rita Bianchi5Roberto Fiocca6Sandra Salvi7Fabrizio Montecucco8Maria Bertolotto9Franco Fais10Mario Salio11Emanuela Barisione12Daniela de Totero13Department Experimental Medicine, University of Genoa, 16132 Genoa, ItalyInterventional Pulmonary Unit, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, 16132 Genoa, ItalyDepartment Internal Medicine and Medical Specialties, University of Genoa, 16132 Genoa, ItalyDepartment Internal Medicine and Centre of Excellence for Biomedical Research, University of Genoa, 16132 Genoa, ItalyMolecular Pathology Unit, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, 16132 Genoa, ItalyAnatomic Pathology Unit, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, 16132 Genoa, ItalyAnatomic Pathology Unit, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, 16132 Genoa, ItalyAnatomic Pathology Unit, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, 16132 Genoa, ItalyDepartment Internal Medicine and Medical Specialties, University of Genoa, 16132 Genoa, ItalyDepartment Internal Medicine and Medical Specialties, University of Genoa, 16132 Genoa, ItalyDepartment Experimental Medicine, University of Genoa, 16132 Genoa, ItalyS.C. Malattie Apparato Respiratorio, Azienda Ospedaliera Nazionale Antonio, Biagio e Cesare Arrigo, 15121 Alessandria, ItalyInterventional Pulmonary Unit, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, 16132 Genoa, ItalyMolecular Pathology Unit, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, 16132 Genoa, ItalyHypersensitivity pneumonitis (HP) is a diffuse interstitial lung disease (ILD) caused by the inhalation of a variety of antigens in susceptible individuals. Patients with fibrotic HP (fHP) may show histopathological and radiological manifestations similar to patients with idiopathic pulmonary fibrosis (usual interstitial pneumonia-like pattern of fibrosis) that are associated with a worse prognosis. We describe here the establishment and characterization of a fibroblastic cell line derived from the broncho-alveolar lavage (BAL) of a patient with fHP, a 53 year old man who presented at our Pneumology Unit with cough and dyspnea. The fHP diagnosis was based on international criteria and multidisciplinary discussion. Primary fibroblasts were expanded in vitro until passage 36. These fibroblasts displayed morpho/phenotypical features of myofibroblasts, showing high positivity for α-smooth muscle actin, type I collagen, and fibronectin as determined by quantitative RT-PCR and cyto-fluorographic analysis. Cytogenetic analyses further evidenced trisomy of chromosome 10, which interestingly harbors the FGF2R gene. To our knowledge, this is the first fibroblastic cell line derived from an fHP patient and might, therefore, represent a suitable tool to model the disease in vitro. We preliminarily assessed here the activity of pirfenidone, further demonstrating a consistent inhibition of cells growth by this antifibrotic drug.https://www.mdpi.com/2227-9059/9/9/1193hypersensitivity pneumonitisfibrosismyofibroblastspirfenidone |
| spellingShingle | Paolo Giannoni Marco Grosso Giuseppina Fugazza Mario Nizzari Maria Cristina Capra Rita Bianchi Roberto Fiocca Sandra Salvi Fabrizio Montecucco Maria Bertolotto Franco Fais Mario Salio Emanuela Barisione Daniela de Totero Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity Pneumonitis Biomedicines hypersensitivity pneumonitis fibrosis myofibroblasts pirfenidone |
| title | Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity Pneumonitis |
| title_full | Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity Pneumonitis |
| title_fullStr | Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity Pneumonitis |
| title_full_unstemmed | Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity Pneumonitis |
| title_short | Establishment and Characterization of a Novel Fibroblastic Cell Line (SCI13D) Derived from the Broncho-Alveolar Lavage of a Patient with Fibrotic Hypersensitivity Pneumonitis |
| title_sort | establishment and characterization of a novel fibroblastic cell line sci13d derived from the broncho alveolar lavage of a patient with fibrotic hypersensitivity pneumonitis |
| topic | hypersensitivity pneumonitis fibrosis myofibroblasts pirfenidone |
| url | https://www.mdpi.com/2227-9059/9/9/1193 |
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