Literature DB >> 34799298

Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip.

Roberto Plebani1, Ratnakar Potla2, Mercy Soong3, Haiqing Bai3, Zohreh Izadifar3, Amanda Jiang3, Renee N Travis3, Chaitra Belgur3, Alexandre Dinis3, Mark J Cartwright3, Rachelle Prantil-Baun3, Pawan Jolly3, Sarah E Gilpin3, Mario Romano4, Donald E Ingber5.   

Abstract

BACKGROUND: Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), which results in impaired airway mucociliary clearance, inflammation, infection, and respiratory insufficiency. The development of new therapeutics for CF are limited by the lack of reliable preclinical models that recapitulate the structural, immunological, and bioelectrical features of human CF lungs.
METHODS: We leveraged organ-on-a-chip technology to develop a microfluidic device lined by primary human CF bronchial epithelial cells grown under an air-liquid interface and interfaced with pulmonary microvascular endothelial cells (CF Airway Chip) exposed to fluid flow. The responses of CF and healthy Airway Chips were analyzed in the presence or absence of polymorphonuclear leukocytes (PMNs) and the bacterial pathogen, Pseudomonas aeruginosa.
RESULTS: The CF Airway Chip faithfully recapitulated many features of the human CF airways, including enhanced mucus accumulation, increased cilia density, and a higher ciliary beating frequency compared to chips lined by healthy bronchial epithelial cells. The CF chips also secreted higher levels of IL-8, which was accompanied by enhanced PMN adhesion to the endothelium and transmigration into the airway compartment. In addition, CF Airway Chips provided a more favorable environment for Pseudomonas aeruginosa growth, which resulted in enhanced secretion of inflammatory cytokines and recruitment of PMNs to the airway.
CONCLUSIONS: The human CF Airway Chip may provide a valuable preclinical tool for pathophysiology studies as well as for drug testing and personalized medicine.
Copyright © 2021 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Cystic fibrosis; Microfluidics; Neutrophils; Organ chip; Pseudomonas

Mesh:

Substances:

Year:  2021        PMID: 34799298     DOI: 10.1016/j.jcf.2021.10.004

Source DB:  PubMed          Journal:  J Cyst Fibros        ISSN: 1569-1993            Impact factor:   5.527


  10 in total

1.  Imaging-guided bioreactor for de-epithelialization and long-term cultivation of ex vivo rat trachea.

Authors:  Seyed Mohammad Mir; Jiawen Chen; Meghan R Pinezich; John D O'Neill; Sarah X L Huang; Gordana Vunjak-Novakovic; Jinho Kim
Journal:  Lab Chip       Date:  2022-03-01       Impact factor: 6.799

Review 2.  What Can an Organ-on-a-Chip Teach Us About Human Lung Pathophysiology?

Authors:  Haiqing Bai; Donald E Ingber
Journal:  Physiology (Bethesda)       Date:  2022-06-06

3.  Imaging-Guided Bioreactor for Generating Bioengineered Airway Tissue.

Authors:  Seyed Mohammad Mir; Jiawen Chen; Meghan R Pinezich; John D O'Neill; Brandon A Guenthart; Gordana Vunjak-Novakovic; Jinho Kim
Journal:  J Vis Exp       Date:  2022-04-06       Impact factor: 1.424

Review 4.  Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics.

Authors:  Deborah M Cholon; Martina Gentzsch
Journal:  Curr Opin Pharmacol       Date:  2022-04-21       Impact factor: 4.768

Review 5.  3D In Vitro Models: Novel Insights into Idiopathic Pulmonary Fibrosis Pathophysiology and Drug Screening.

Authors:  Ana Ivonne Vazquez-Armendariz; Margarida Maria Barroso; Elie El Agha; Susanne Herold
Journal:  Cells       Date:  2022-05-02       Impact factor: 7.666

6.  Homogeneous Distribution of Exogenous Cells onto De-epithelialized Rat Trachea via Instillation of Cell-Loaded Hydrogel.

Authors:  Jiawen Chen; Seyed Mohammad Mir; Meghan R Pinezich; John D O'Neill; Brandon A Guenthart; Matthew Bacchetta; Gordana Vunjak-Novakovic; Sarah X L Huang; Jinho Kim
Journal:  ACS Biomater Sci Eng       Date:  2021-12-07

Review 7.  Engineering Organoids for in vitro Modeling of Phenylketonuria.

Authors:  Alice C Borges; Kerensa Broersen; Paula Leandro; Tiago G Fernandes
Journal:  Front Mol Neurosci       Date:  2022-01-10       Impact factor: 5.639

Review 8.  3D Lung Tissue Models for Studies on SARS-CoV-2 Pathophysiology and Therapeutics.

Authors:  Roberto Plebani; Haiqing Bai; Longlong Si; Jing Li; Chunhe Zhang; Mario Romano
Journal:  Int J Mol Sci       Date:  2022-09-03       Impact factor: 6.208

Review 9.  Advances in Preclinical In Vitro Models for the Translation of Precision Medicine for Cystic Fibrosis.

Authors:  Iris A L Silva; Onofrio Laselva; Miquéias Lopes-Pacheco
Journal:  J Pers Med       Date:  2022-08-16

Review 10.  Lung-Directed Bacteriotherapy in Cystic Fibrosis: Could It Be an Option?

Authors:  Giovanna Batoni; Giuseppantonio Maisetta; Esingül Kaya; Semih Esin
Journal:  Antibiotics (Basel)       Date:  2022-02-28
  10 in total

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