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We are analyzing https://www.nature.com/articles/s41467-019-11327-1.

Title:
Mitofusins regulate lipid metabolism to mediate the development of lung fibrosis | Nature Communications
Description:
Accumulating 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. Mitochondria of alveolar type 2 epithelial cells (AEC2) in the lung have been suggested to play a role in the development of idiopathic pulmonary fibrosis (IPF). Here the authors show that loss of mitofusin1 and mitofusin2 in murine AEC2 cells leads to the development of lung fibrosis through the regulation of surfactant lipids.
Website Age:
30 years and 10 months (reg. 1994-08-11).

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Keywords {๐Ÿ”}

cells, aec, mfn, mice, mitochondrial, lung, fig, fibrosis, cell, bleomycin, article, lipid, mfniฮดaec, google, scholar, supplementary, control, data, treatment, cas, metabolism, synthesis, sftpccreert, murine, genes, staining, lungs, nature, mitochondria, surfactant, deletion, development, pulmonary, analysis, fusion, metabolic, purine, pbs, group, performed, mitophagy, tem, source, damage, role, loss, acid, bleomycininduced, regulation, days,

Topics {โœ’๏ธ}

nature portfolio org/packages/release/bioc/html/limma privacy policy org/packages/release/bioc/html/clusterprofiler /software/tophat/index retroviral construct pchac-mt-mkeima advertising fitc-conjugated anti-biotin antibody nature communications high-throughput cre reporting phosphatidylinositol-3-phosphate regulates sorting io/cufflinks/ social media aec2 cell-specific sftpc-promoter source data file bmj open respir translational research program reprints full size image scientific advisory board medical research atf4-mediated stress pathways richard allan scientific single-cell rna-seq tamoxifen-inducible tdtomato labeling reporting summary transforming growth factor-beta1 nature 507 nature 524 nature 429 nature 509 nature 456 nature research design tamoxifen-inducible tdtomato florescence library construction fatty acid synthase author information authors bleomycin-induced lung fibrosis biotin-conjugated anti-epcam intra-tracheal administration kaplanโ€“meier survival curves profibrotic tgf-beta signaling tamoxifen-inducible tdtomato fluorescence acid-soluble collagen depositions tamoxifen-inducible fasn deletion tamoxifen-inducible fasn knockout adenosine-dependent pulmonary fibrosis acid-soluble collagen showed aec2 cell-specific deletion

Schema {๐Ÿ—บ๏ธ}

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      headline:Mitofusins regulate lipid metabolism to mediate the development of lung fibrosis
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      name:Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan
      name:Department of Medical Research, National Taiwan University Hospital, Taipei, Taiwan
      name:Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
      name:Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
      name:Division of Nephrology and Hypertension, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
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      name:Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
      name:Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
      name:Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
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      name:Department of Internal Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan
      name:Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
      name:Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, USA
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