The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.

<h4>Background</h4>We previously identified a MUC5B gene promoter-variant that is a risk allele for sporadic and familial Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia (IPF/UIP). This allele was strongly associated with increased MUC5B gene expression in lung tissue from una...

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Autores principales:	Max A Seibold, Russell W Smith, Cydney Urbanek, Steve D Groshong, Gregory P Cosgrove, Kevin K Brown, Marvin I Schwarz, David A Schwartz, Susan D Reynolds
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2013
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/46b6c964ccf44a8cb7bf503b84370458
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spelling	oai:doaj.org-article:46b6c964ccf44a8cb7bf503b843704582021-11-18T07:52:43ZThe idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.1932-620310.1371/journal.pone.0058658https://doaj.org/article/46b6c964ccf44a8cb7bf503b843704582013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/23527003/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Background</h4>We previously identified a MUC5B gene promoter-variant that is a risk allele for sporadic and familial Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia (IPF/UIP). This allele was strongly associated with increased MUC5B gene expression in lung tissue from unaffected subjects. Despite the strong association of this airway epithelial marker with disease, little is known of mucin expressing structures or of airway involvement in IPF/UIP.<h4>Methods</h4>Immunofluorescence was used to subtype mucus cells according to MUC5B and MUC5AC expression and to identify ciliated, basal, and alveolar type II (ATII) cells in tissue sections from control and IPF/UIP subjects. Staining patterns were quantified for distal airways (Control and IPF/UIP) and in honeycomb cysts (HC).<h4>Results</h4>MUC5B-expressing cells (EC) were detected in the majority of control distal airways. MUC5AC-EC were identified in half of these airways and only in airways that contained MUC5B-EC. The frequency of MUC5B+ and MUC5AC+ distal airways was increased in IPF/UIP subjects. MUC5B-EC were the dominant mucus cell type in the HC epithelium. The distal airway epithelium from control and IPF/UIP subjects and HC was populated by basal and ciliated cells. Most honeycombing regions were distinct from ATII hyperplasic regions. ATII cells were undetectable in the overwhelming majority of HC.<h4>Conclusions</h4>The distal airway contains a pseudostratified mucocilary epithelium that is defined by basal epithelial cells and mucus cells that express MUC5B predominantly. These data suggest that the HC is derived from the distal airway.Max A SeiboldRussell W SmithCydney UrbanekSteve D GroshongGregory P CosgroveKevin K BrownMarvin I SchwarzDavid A SchwartzSusan D ReynoldsPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 3, p e58658 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Max A Seibold Russell W Smith Cydney Urbanek Steve D Groshong Gregory P Cosgrove Kevin K Brown Marvin I Schwarz David A Schwartz Susan D Reynolds The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
description	<h4>Background</h4>We previously identified a MUC5B gene promoter-variant that is a risk allele for sporadic and familial Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia (IPF/UIP). This allele was strongly associated with increased MUC5B gene expression in lung tissue from unaffected subjects. Despite the strong association of this airway epithelial marker with disease, little is known of mucin expressing structures or of airway involvement in IPF/UIP.<h4>Methods</h4>Immunofluorescence was used to subtype mucus cells according to MUC5B and MUC5AC expression and to identify ciliated, basal, and alveolar type II (ATII) cells in tissue sections from control and IPF/UIP subjects. Staining patterns were quantified for distal airways (Control and IPF/UIP) and in honeycomb cysts (HC).<h4>Results</h4>MUC5B-expressing cells (EC) were detected in the majority of control distal airways. MUC5AC-EC were identified in half of these airways and only in airways that contained MUC5B-EC. The frequency of MUC5B+ and MUC5AC+ distal airways was increased in IPF/UIP subjects. MUC5B-EC were the dominant mucus cell type in the HC epithelium. The distal airway epithelium from control and IPF/UIP subjects and HC was populated by basal and ciliated cells. Most honeycombing regions were distinct from ATII hyperplasic regions. ATII cells were undetectable in the overwhelming majority of HC.<h4>Conclusions</h4>The distal airway contains a pseudostratified mucocilary epithelium that is defined by basal epithelial cells and mucus cells that express MUC5B predominantly. These data suggest that the HC is derived from the distal airway.
format	article
author	Max A Seibold Russell W Smith Cydney Urbanek Steve D Groshong Gregory P Cosgrove Kevin K Brown Marvin I Schwarz David A Schwartz Susan D Reynolds
author_facet	Max A Seibold Russell W Smith Cydney Urbanek Steve D Groshong Gregory P Cosgrove Kevin K Brown Marvin I Schwarz David A Schwartz Susan D Reynolds
author_sort	Max A Seibold
title	The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
title_short	The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
title_full	The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
title_fullStr	The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
title_full_unstemmed	The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
title_sort	idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
publisher	Public Library of Science (PLoS)
publishDate	2013
url	https://doaj.org/article/46b6c964ccf44a8cb7bf503b84370458
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The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.

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