{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T21:08:28Z","timestamp":1793048908396,"version":"4.5.0"},"reference-count":57,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2025,10,15]],"date-time":"2025-10-15T00:00:00Z","timestamp":1760486400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["P01-HL162607"],"award-info":[{"award-number":["P01-HL162607"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01-HL097163"],"award-info":[{"award-number":["R01-HL097163"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01-HL158668"],"award-info":[{"award-number":["R01-HL158668"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["P01-HL092870"],"award-info":[{"award-number":["P01-HL092870"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["UH3-HL123442"],"award-info":[{"award-number":["UH3-HL123442"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["UG3-HL151865"],"award-info":[{"award-number":["UG3-HL151865"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Veterans Affairs Merit Review","award":["IO1BX005295"],"award-info":[{"award-number":["IO1BX005295"]}]}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,5,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Rationale: IPF is an irreversible and progressive type of lung fibrosis that is pathologically characterized as spatially heterogeneous. Despite the identified dominant risk factor for IPF as the gain-of-function MUC5B promoter variant, little is understood for how MUC5B drives lung fibrosis.<\/jats:p>\n                  <jats:p>Objectives: We used spatial transcriptomics from idiopathic pulmonary fibrosis (IPF) and unaffected control lung tissue to further understand the pathogenesis of MUC5B-driven lung fibrosis.<\/jats:p>\n                  <jats:p>Methods: We captured 43 fields of view in 15 IPF and 13 controls with and without the MUC5B promoter variant using the CosMx\u00ae platform and identified 19 cell types via semi-supervised clustering.<\/jats:p>\n                  <jats:p>Measurements and Main Results: \u00a0MUC5B was ectopically expressed in AT2 cells in controls with the risk variant. We observed a decreased proportion of AT2 cells in controls and an increased proportion of aberrant basaloid cells in IPF associated with the MUC5B risk variant. We identified co-localized expression of MUC5B in respiratory bronchioles with 13 genes including the endoplasmic reticulum (ER) stress marker XBP1 and distal secretory markers SCGB3A1 and SCGB1A1. Experimentally, we demonstrated a direct relationship between MUC5B expression and ER stress in bronchiolar epithelia in vitro and validated the co-expression of MUC5B and XBP1 in the IPF lung.<\/jats:p>\n                  <jats:p>Conclusions: Based on our results, we conclude that MUC5B injures alveolar and bronchiolar epithelia that results in loss of AT2 cells and an increase in aberrant basaloid cells which initiates ER stress and a secretory phenotype in the terminal respiratory bronchiole, establishing a persistently injured distal airspace.<\/jats:p>","DOI":"10.1165\/rcmb.2025-0261oc","type":"journal-article","created":{"date-parts":[[2025,10,15]],"date-time":"2025-10-15T14:21:02Z","timestamp":1760538062000},"page":"632-643","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":4,"title":["Ectopic expression of\n                    <i>MUC5B<\/i>\n                    in the respiratory bronchiole initiates endoplasmic reticulum stress in the IPF lung"],"prefix":"10.1093","volume":"74","author":[{"given":"Rachel Z","family":"Blumhagen","sequence":"first","affiliation":[{"name":"Center for Genes, Environment and Health, National Jewish Health , Denver, CO 80206,","place":["United States"]},{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan S","family":"Kurche","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]},{"name":"Rocky Mountain Regional Veteran\u2019s Administration Medical Center , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlyne D","family":"Cool","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]},{"name":"Pathology Laboratory, National Jewish Health , Denver, CO 80206,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Heinz","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, National Jewish Health , Denver, CO 80206,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoqian","family":"Ma","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth J","family":"Davidson","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]},{"name":"Department of Biomedical Informatics, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tasha E","family":"Fingerlin","sequence":"additional","affiliation":[{"name":"Center for Genes, Environment and Health, National Jewish Health , Denver, CO 80206,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan P","family":"Huber","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evgenia","family":"Dobrinskikh","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]},{"name":"Department of Pediatrics, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A","family":"Schwartz","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]},{"name":"Rocky Mountain Regional Veteran\u2019s Administration Medical Center , Aurora, CO 80045,","place":["United States"]},{"name":"Department of Immunology and Microbiology, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivana V","family":"Yang","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]},{"name":"Department of Biomedical Informatics, University of Colorado Anschutz Medical Campus , Aurora, CO 80045,","place":["United States"]},{"name":"Department of Epidemiology, Colorado School of Public Health , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,10,15]]},"reference":[{"key":"2026082311065245700_aanaf031-B1","doi-asserted-by":"crossref","first-page":"e44","DOI":"10.1164\/rccm.201807-1255ST","article-title":"Diagnosis of idiopathic pulmonary fibrosis. 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