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Editorial
. 2022 Aug;67(2):142-144.
doi: 10.1165/rcmb.2022-0201ED.

Aberrant Multiciliogenesis in Pulmonary Fibrosis: Bystander or Driver of Disease Progression?

Mada Ghanem  1 , Arnaud A Mailleux  1
Affiliations
Editorial

Aberrant Multiciliogenesis in Pulmonary Fibrosis: Bystander or Driver of Disease Progression?

Mada Ghanem et al. Am J Respir Cell Mol Biol. 2022 Aug.
No abstract available

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Figures

Figure 1.
Figure 1.
Aberrant multiciliogenesis and bronchiolization of distal airways in idiopathic pulmonary fibrosis (IPF). Proposed model for the involvement of primary cilium (IFT88), SHH (Sonic Hedgehog) signaling in basal cells leading to KRT5+ pod formation and aberrant multiciliogenesis after injury. The insert on the left showed immunofluorescence of a primary cilium from an IPF lung section. The axonem of the cilium was labeled with acetylated tubulin (red) and the basal body with pericentrin (green); nuclei (blue) were counterstained with2-(4-Amidinophenyl)-6-indolecarbamidine dihydrochloride (DAPI). Defective mucociliary clearance (MCC) as well as perturbed interactions between abnormal multiciliated epithelial cells (see acetylated tubulin immunofluorescence labeling the cilia in an IPF lung section; nuclei [blue] are counterstained with DAPI) and fibroblasts (leading to fibroproliferation) would promote fibrosis and bronchiolization in distal lung airways (as shown in the hematoxylin and eosin staining on the right). (Scale bar, 10 μm [left panel], 25 μm [middle panel], and 50 μm [right panel].)
See this image and copyright information in PMC

Comment on

  • Aberrant Multiciliogenesis in Idiopathic Pulmonary Fibrosis.
    Kim E, Mathai SK, Stancil IT, Ma X, Hernandez-Gutierrez A, Becerra JN, Marrero-Torres E, Hennessy CE, Hatakka K, Wartchow EP, Estrella A, Huber JP, Cardwell JH, Burnham EL, Zhang Y, Evans CM, Vladar EK, Schwartz DA, Dobrinskikh E, Yang IV. Kim E, et al. Am J Respir Cell Mol Biol. 2022 Aug;67(2):188-200. doi: 10.1165/rcmb.2021-0554OC. Am J Respir Cell Mol Biol. 2022. PMID: 35608953 Free PMC article.

References

    1. Froidure A, Marchal-Duval E, Homps-Legrand M, Ghanem M, Justet A, Crestani B, et al. Chaotic activation of developmental signaling pathways drives idiopathic pulmonary fibrosis. Eur Respir Rev . 2020;29:190140. - PMC - PubMed
    1. Hancock LA, Hennessy CE, Solomon GM, Dobrinskikh E, Estrella A, Hara N, et al. Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice. Nat Commun . 2018;9:5363. - PMC - PubMed
    1. Helling BA, Gerber AN, Kadiyala V, Sasse SK, Pedersen BS, Sparks L, et al. Regulation of MUC5B expression in idiopathic pulmonary fibrosis. Am J Respir Cell Mol Biol . 2017;57:91–99. - PMC - PubMed
    1. Plantier L, Crestani B, Wert SE, Dehoux M, Zweytick B, Guenther A, et al. Ectopic respiratory epithelial cell differentiation in bronchiolised distal airspaces in idiopathic pulmonary fibrosis. Thorax . 2011;66:651–657. - PubMed
    1. Fernanda de Mello Costa M, Weiner AI, Vaughan AE. Basal-like progenitor cells: a review of dysplastic alveolar regeneration and remodeling in lung repair. Stem Cell Reports . 2020;15:1015–1025. - PMC - PubMed