Impact of primary ciliary dyskinesia: Beyond sinobronchial syndrome in Japan

N Keicho, M Hijikata, A Miyabayashi… - Respiratory …, 2024 - Elsevier
Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by impaired motile
cilia function, particularly in the upper and lower airways. To date, more than 50 causative …

Nontuberculous Mycobacteria, Mucociliary Clearance, and Bronchiectasis

M Retuerto-Guerrero, R López-Medrano… - Microorganisms, 2024 - mdpi.com
Nontuberculous mycobacteria (NTM) are environmental and ubiquitous, but only a few
species are associated with disease, often presented as nodular/bronchiectatic or cavitary …

[HTML][HTML] Loss of an extensive ciliary connectome induces proteostasis and cell fate switching in a severe motile ciliopathy

SL Brody, J Pan, T Huang, J Xu, H Xu, J Koenitizer… - bioRxiv, 2024 - ncbi.nlm.nih.gov
Motile cilia have essential cellular functions in development, reproduction, and homeostasis.
Genetic causes for motile ciliopathies have been identified, but the consequences on …

RBL2 represses the transcriptional activity of Multicilin to inhibit multiciliogenesis

EJ Quiroz, S Kim, LK Gautam, Z Borok, C Kintner… - Cell death & …, 2024 - nature.com
A core pathophysiologic feature underlying many respiratory diseases is multiciliated cell
dysfunction, leading to inadequate mucociliary clearance. Due to the prevalence and highly …

Gene dosage of independent dynein arm motor preassembly factors influences cilia assembly in Chlamydomonas reinhardtii

GM Penny, SK Dutcher - PLoS genetics, 2024 - journals.plos.org
Motile cilia assembly utilizes over 800 structural and cytoplasmic proteins. Variants in
approximately 58 genes cause primary ciliary dyskinesia (PCD) in humans, including the …

Reduced dosage of multiple dynein arm preassembly factors limits cilia regeneration in Chlamydomonas reinhardtii

GM Penny, SK Dutcher - bioRxiv, 2023 - biorxiv.org
Motile cilia are complex organelles comprised of> 800 structural proteins. Variants in 58 of
these genes cause primary ciliary dyskinesia (PCD) in humans. We used a second-site non …