Multicellular modeling of ciliopathy by combining iPS cells and microfluidic airway-on-a-chip technology
N Sone, S Konishi, K Igura, K Tamai, S Ikeo… - Science translational …, 2021 - science.org
Mucociliary clearance is an essential lung function that facilitates the removal of inhaled
pathogens and foreign matter unidirectionally from the airway tract and is innately achieved …
pathogens and foreign matter unidirectionally from the airway tract and is innately achieved …
Induced pluripotent stem cells for primary ciliary dyskinesia modeling and personalized medicine
J Mianné, E Ahmed, C Bourguignon… - American journal of …, 2018 - atsjournals.org
Primary ciliary dyskinesia (PCD) is a rare and heterogeneous genetic disorder that affects
the structure and function of motile cilia. In the airway epithelium, impaired ciliary motion …
the structure and function of motile cilia. In the airway epithelium, impaired ciliary motion …
[HTML][HTML] The Cilialyzer–A freely available open-source software for the analysis of mucociliary activity in respiratory cells
M Schneiter, SA Tschanz, A Escher, L Müller… - Computer methods and …, 2023 - Elsevier
Abstract Background and Objective Primary ciliary dyskinesia (PCD) is a rare genetic
disorder causing a defective ciliary structure, which predominantly leads to an impaired …
disorder causing a defective ciliary structure, which predominantly leads to an impaired …
[HTML][HTML] Airway epithelial homeostasis and planar cell polarity signaling depend on multiciliated cell differentiation
EK Vladar, JV Nayak, CE Milla, JD Axelrod - JCI insight, 2016 - ncbi.nlm.nih.gov
Motile airway cilia that propel contaminants out of the lung are oriented in a common
direction by planar cell polarity (PCP) signaling, which localizes PCP protein complexes to …
direction by planar cell polarity (PCP) signaling, which localizes PCP protein complexes to …
Modelling ciliopathy phenotypes in human tissues derived from pluripotent stem cells with genetically ablated cilia
NM Cruz, R Reddy, JL McFaline-Figueroa… - Nature biomedical …, 2022 - nature.com
The functions of cilia—antenna-like organelles associated with a spectrum of disease states—
are poorly understood, particularly in human cells. Here we show that human pluripotent …
are poorly understood, particularly in human cells. Here we show that human pluripotent …
Motile cilia and airway disease
M Legendre, LE Zaragosi, HM Mitchison - Seminars in cell & …, 2021 - Elsevier
A finely regulated system of airway epithelial development governs the differentiation of
motile ciliated cells of the human respiratory tract, conferring the body's mucociliary …
motile ciliated cells of the human respiratory tract, conferring the body's mucociliary …
Observing planar cell polarity in multiciliated mouse airway epithelial cells
The concerted movement of cilia propels inhaled contaminants out of the lungs,
safeguarding the respiratory system from toxins, pathogens, pollutants, and allergens. Motile …
safeguarding the respiratory system from toxins, pathogens, pollutants, and allergens. Motile …
A quantitative super-resolution imaging toolbox for diagnosis of motile ciliopathies
Airway clearance of pathogens and particulates relies on motile cilia. Impaired cilia motility
can lead to reduction in lung function, lung transplant, or death in some cases. More than 50 …
can lead to reduction in lung function, lung transplant, or death in some cases. More than 50 …
Modelling of primary ciliary dyskinesia using patient‐derived airway organoids
J Van der Vaart, L Böttinger, MH Geurts… - EMBO …, 2021 - embopress.org
Patient‐derived human organoids can be used to model a variety of diseases. Recently, we
described conditions for long‐term expansion of human airway organoids (AOs) directly …
described conditions for long‐term expansion of human airway organoids (AOs) directly …
Active mucus–cilia hydrodynamic coupling drives self-organization of human bronchial epithelium
The respiratory tract is protected by mucus, a complex fluid transported along the epithelial
surface by the coordinated beating of millions of microscopic cilia, hence the name of …
surface by the coordinated beating of millions of microscopic cilia, hence the name of …