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Due to its involvement in extracellular matrix (ECM) remodelling, myofibroblast differentiation and apoptosis, HSP 90 has been investigated as a key player in the pathogenesis of lung fibrosis. Idiopathic pulmonary fibrosis (IPF) is the most common and deadly interstitial lung disease, due to the progressive distortion of lung parenchyma related to the overproduction and deposition of altered ECM, driven by transforming growth factor-\u03b2 (TGF-\u03b2) dependent and independent pathways. The inhibition or induction of HSP 90 is associated with a reduced or increased expression of TGF-\u03b2 receptors, respectively, suggesting a role for HSP 90 as a biomarker and therapeutic target in IPF. Experimental drugs such as geldanamycin and its derivatives 17-AAG (17-<jats:italic>N<\/jats:italic>-allylamino-17-demethoxygeldanamicin) and 17-DMAG (17-dimethylaminoethylamino-17-demethoxigeldanamycin), along with AUY-922, 1G6-D7, AT-13387, TAS-116 and myricetin, have been found to reduce lung fibrosis in both<jats:italic>in vivo<\/jats:italic>and<jats:italic>in vitro<\/jats:italic>models, supporting the role of this emerging target. This review aims to illustrate the structure and biological function of HSP 90 in the context of IPF pathobiology, as well as perspective application of this molecule as a biomarker and therapeutic target for IPF.<\/jats:p>","DOI":"10.1183\/16000617.0147-2024","type":"journal-article","created":{"date-parts":[[2025,3,21]],"date-time":"2025-03-21T07:34:53Z","timestamp":1742542493000},"page":"240147","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":8,"title":["The role of heat shock protein 90 in idiopathic pulmonary fibrosis: state of the art"],"prefix":"10.1183","volume":"34","author":[{"given":"Giorgio","family":"Monteleone","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paolo","family":"Cameli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7579-9767","authenticated-orcid":false,"given":"Francesco","family":"Bonella","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"81","published-online":{"date-parts":[[2025,3,19]]},"reference":[{"key":"2025032100334829000_34.175.240147.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2007.04.030"},{"key":"2025032100334829000_34.175.240147.2","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1002\/path.5349","article-title":"Molecular mechanisms in chaperonopathies: clues to understanding the histopathological abnormalities and developing novel therapies","volume":"250","author":"Macario","year":"2020","journal-title":"J Pathol"},{"key":"2025032100334829000_34.175.240147.3","doi-asserted-by":"crossref","first-page":"9316","DOI":"10.3390\/ijms22179316","article-title":"Extracellular heat shock proteins as therapeutic targets and biomarkers in fibrosing interstitial lung diseases","volume":"22","author":"Tanguy","year":"2021","journal-title":"Int J Mol Sci"},{"key":"2025032100334829000_34.175.240147.4","doi-asserted-by":"publisher","DOI":"10.1016\/j.canlet.2010.10.014"},{"key":"2025032100334829000_34.175.240147.5","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-017-00449-z"},{"key":"2025032100334829000_34.175.240147.6","doi-asserted-by":"publisher","DOI":"10.1038\/s41419-022-05373-9"},{"key":"2025032100334829000_34.175.240147.7","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.REV118.002806"},{"key":"2025032100334829000_34.175.240147.8","doi-asserted-by":"publisher","DOI":"10.3390\/ijms19092560"},{"key":"2025032100334829000_34.175.240147.9","doi-asserted-by":"crossref","first-page":"2846","DOI":"10.3390\/molecules23112846","article-title":"Heat shock proteins as immunomodulants","volume":"23","author":"Zininga","year":"2018","journal-title":"Molecules"},{"key":"2025032100334829000_34.175.240147.10","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(17)30866-8"},{"key":"2025032100334829000_34.175.240147.11","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1705751"},{"key":"2025032100334829000_34.175.240147.12","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00957-2022"},{"key":"2025032100334829000_34.175.240147.13","doi-asserted-by":"publisher","DOI":"10.1021\/pr1009355"},{"key":"2025032100334829000_34.175.240147.14","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01941-2015"},{"key":"2025032100334829000_34.175.240147.15","doi-asserted-by":"publisher","DOI":"10.1016\/j.pharmthera.2014.02.009"},{"key":"2025032100334829000_34.175.240147.16","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00386-2017"},{"key":"2025032100334829000_34.175.240147.17","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.16.9.4691"},{"key":"2025032100334829000_34.175.240147.18","doi-asserted-by":"crossref","first-page":"106","DOI":"10.4103\/2319-4170.113230","article-title":"Function and regulation of the Hsp90 machinery","volume":"36","author":"Buchner","year":"2013","journal-title":"Biomed J"},{"key":"2025032100334829000_34.175.240147.19","doi-asserted-by":"publisher","DOI":"10.1038\/nrm.2017.20"},{"key":"2025032100334829000_34.175.240147.20","doi-asserted-by":"publisher","DOI":"10.1038\/nrc1716"},{"key":"2025032100334829000_34.175.240147.21","doi-asserted-by":"crossref","unstructured":"Jackson SE . 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