Mitofusins Regulate Lipid Metabolism to Mediate the Development of Lung Fibrosis

Published: 14 Jun 2019 | Version 3 | DOI: 10.17632/29v5w97mx4.3
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Description of this data

Mitochondria form a dynamic intracellular network through fusion and fission to regulate cellular metabolism. Accumulating evidence illustrates a fundamental role for mitochondria in lung alveolar type 2 epithelial cell (AEC2) dysfunction in the pathogenesis of idiopathic pulmonary fibrosis (IPF). In this study, 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 surfactant lipid metabolism, where loss of MFN1, MFN2 or inhibiting lipid synthesis via fatty acid synthase (FASN) deficiency exacerbates bleomycin-induced lung fibrosis. We demonstrate that AEC2 cell lipid metabolism confers a protective and adaptive response against lung fibrosis and propose a novel tenet that mitochondrial fusion and lipid metabolism are tightly linked to regulate AEC2 cell injury and subsequent fibrotic remodeling in the lung.

Experiment data files

Latest version

  • Version 3

    2019-06-14

    Published: 2019-06-14

    DOI: 10.17632/29v5w97mx4.3

    Cite this dataset

    Chung, Kuei-Pin; Cloonan, Suzanne; Choi, Augustine (2019), “Mitofusins Regulate Lipid Metabolism to Mediate the Development of Lung Fibrosis”, Mendeley Data, v3 http://dx.doi.org/10.17632/29v5w97mx4.3

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Categories

Lipid Metabolism, Mitochondrion, Pulmonary Fibrosis, Alveolar Type II Cells

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This dataset is licensed under a Creative Commons Attribution 4.0 International licence. What does this mean? You can share, copy and modify this dataset so long as you give appropriate credit, provide a link to the CC BY license, and indicate if changes were made, but you may not do so in a way that suggests the rights holder has endorsed you or your use of the dataset. Note that further permission may be required for any content within the dataset that is identified as belonging to a third party.

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