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dc.contributor.authorCloonan, Suzanne
dc.date.accessioned2021-07-13T14:36:32Z
dc.date.available2021-07-13T14:36:32Z
dc.date.issued2019
dc.date.submitted2019en
dc.identifier.citationChung, Kuei-Pin and Hsu, Chia-Lang and Fan, Li-Chao and Huang, Ziling and Bhatia, Divya and Chen, Yi-Jung and Hisata, Shu and Cho, Soo Jung and Nakahira, Kiichi and Imamura, Mitsuru and Choi, Mary E and Yu, Chong-Jen and Cloonan, Suzanne M and Choi, Augustine M K, Mitofusins regulate lipid metabolism to mediate the development of lung fibrosis, Nature communications, 2019, 10, 1, 3390en
dc.identifier.issn2041-1723
dc.identifier.otherY
dc.identifier.urihttp://hdl.handle.net/2262/96738
dc.descriptionPUBLISHEDen
dc.description.abstractAccumulating evidence illustrates a fundamental role for mitochondria in lung alveolar type 2 epithelial cell (AEC2) dysfunction in the pathogenesis of idiopathic pulmonary fibrosis. However, the role of mitochondrial fusion in AEC2 function and lung fibrosis development remains unknown. Here we report that the absence of the mitochondrial fusion proteins mitofusin1 (MFN1) and mitofusin2 (MFN2) in murine AEC2 cells leads to morbidity and mortality associated with spontaneous lung fibrosis. We uncover a crucial role for MFN1 and MFN2 in the production of surfactant lipids with MFN1 and MFN2 regulating the synthesis of phospholipids and cholesterol in AEC2 cells. Loss of MFN1, MFN2 or inhibiting lipid synthesis via fatty acid synthase deficiency in AEC2 cells exacerbates bleomycin-induced lung fibrosis. We propose a tenet that mitochondrial fusion and lipid metabolism are tightly linked to regulate AEC2 cell injury and subsequent fibrotic remodeling in the lung.en
dc.format.extent3390en
dc.language.isoenen
dc.relation.ispartofseriesNature communications;
dc.relation.ispartofseries10;
dc.relation.ispartofseries1;
dc.rightsYen
dc.subjectAlveolar Epithelial Cellsen
dc.subjectAnimalsen
dc.subjectAntibioticsen
dc.subjectMitochondriaen
dc.subjectRespiratory tract diseasesen
dc.subjectLipidsen
dc.titleMitofusins regulate lipid metabolism to mediate the development of lung fibrosisen
dc.typeJournal Articleen
dc.type.supercollectionscholarly_publicationsen
dc.type.supercollectionrefereed_publicationsen
dc.identifier.peoplefinderurlhttp://people.tcd.ie/cloonas
dc.identifier.rssinternalid217585
dc.identifier.doihttp://dx.doi.org/10.1038/s41467-019-11327-1
dc.rights.ecaccessrightsopenAccess
dc.identifier.orcid_id0000-0001-5301-9926


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