{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T17:36:19Z","timestamp":1791999379794,"version":"4.4.0"},"reference-count":62,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T00:00:00Z","timestamp":1732492800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82370492, 82170504, 81730015, 81974048"],"award-info":[{"award-number":["82370492, 82170504, 81730015, 81974048"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81800296"],"award-info":[{"award-number":["81800296"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Fundamental Research Funds"},{"name":"Central Universities","award":["2021GCRC037"],"award-info":[{"award-number":["2021GCRC037"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,3,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background and Aims<\/jats:title>\n                  <jats:p>Marfan syndrome (MFS) is a hereditary disorder primarily caused by mutations in the FBN1 gene. Its critical cardiovascular manifestation is thoracic aortic aneurysm (TAA), which poses life-threatening risks. Owing to the lack of effective pharmacological therapies, surgical intervention continues to be the current definitive treatment. In this study, the role of Piezo-type mechanosensitive ion channel component 1 (Piezo1) in MFS was investigated and the activation of PIEZO1 was identified as a potential treatment for MFS.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>PIEZO1 expression was detected in MFS mice (Fbn1C1041G\/+) and patients. Piezo1 conditional knockout mice in vascular smooth muscle cells of MFS mice (MFS \u00d7 CKO) was generated, and bioinformatics analysis and experiments in vitro and in vivo were performed to investigate the role of Piezo1 in MFS.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>PIEZO1 expression decreased in the aortas of MFS mice; MFS \u00d7 CKO mice showed aggravated TAA, inflammation, extracellular matrix remodelling, and TGF-\u03b2 pathway activation compared to MFS mice. Mechanistically, PIEZO1 knockout exacerbated the activation of the TGF-\u03b2 signalling pathway by inhibiting the endocytosis and autophagy of TGF-\u03b2 receptor 2 mediated by Rab GTPase 3C. Additionally, the pharmacological activation PIEZO1 through Yoda1 prevented TGF-\u03b2 signalling pathway activation and reversed TAA in MFS mice.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Piezo1 deficiency aggravates MFS aneurysms by promoting TGF-\u03b2 signalling pathway activation via TGF-\u03b2 receptor 2 endocytosis and a decrease in autophagy. These data suggest that PIEZO1 may be a potential therapeutic target for MFS treatment.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/eurheartj\/ehae786","type":"journal-article","created":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T16:21:05Z","timestamp":1732551665000},"page":"958-974","source":"Crossref","is-referenced-by-count":26,"title":["PIEZO1 attenuates Marfan syndrome aneurysm development through TGF-\u03b2 signaling pathway inhibition via TGFBR2"],"prefix":"10.1093","volume":"46","author":[{"given":"Peiwen","family":"Yang","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology , 1277 Jiefang Avenue, Wuhan 430022 ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6918-7510","authenticated-orcid":false,"given":"Hao","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Vascular Surgery, Traditional Chinese and Western Medicine Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology , Wuhan ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8738-403X","authenticated-orcid":false,"given":"Shilin","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology , 1277 Jiefang Avenue, Wuhan 430022 ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyue","family":"Xiao","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery, Xiangya Hospital, Central South University , Changsha, Hunan ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lang","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology , 1277 Jiefang Avenue, Wuhan 430022 ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4757-3466","authenticated-orcid":false,"given":"Sheng","family":"Le","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology , 1277 Jiefang Avenue, Wuhan 430022 ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanshan","family":"Chen","sequence":"additional","affiliation":[{"name":"Key Laboratory for Molecular Diagnosis of Hubei Province, Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology , Wuhan ,","place":["China"]},{"name":"Central Laboratory, Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology , Wuhan ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-6812","authenticated-orcid":false,"given":"Ping","family":"Ye","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology , ShengLi Street 26, Wuhan 430014 ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2144-7525","authenticated-orcid":false,"given":"Jiahong","family":"Xia","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology , 1277 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