Ontology highlight
ABSTRACT:
SUBMITTER: Chung KP
PROVIDER: S-EPMC6662701 | biostudies-literature | 2019 Jul
REPOSITORIES: biostudies-literature
Nature communications 20190729 1
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 un ...[more]