title: Mitochondrial UPR: A Double-Edged Sword.
abstract: The mitochondrial unfolded protein response (UPR(mt)) promotes the recovery of dysfunctional mitochondria. Surprisingly, UPR(mt) activation inadvertently maintains and propagates the deleterious mtDNA in a heteroplasmic Caenorhabditis elegans strain, with detrimental consequences. This study extends our understanding of the UPR(mt) and provides a possible therapeutic target for diseases associated with mtDNA mutations.
authors.affiliation: Howard Hughes Medical Institute and Department of Molecular and Cell Biology, Glenn Center for Aging Research, University of California, Berkeley, Berkeley, CA 94720, USA.
authors.forename: Ye
authors.initials: Y
authors.lastname: Tian
authors.affiliation: Howard Hughes Medical Institute and Department of Molecular and Cell Biology, Glenn Center for Aging Research, University of California, Berkeley, Berkeley, CA 94720, USA.
authors.forename: Carsten
authors.initials: C
authors.lastname: Merkwirth
authors.affiliation: Howard Hughes Medical Institute and Department of Molecular and Cell Biology, Glenn Center for Aging Research, University of California, Berkeley, Berkeley, CA 94720, USA. Electronic address: dillin@berkeley.edu.
authors.forename: Andrew
authors.initials: A
authors.lastname: Dillin
mesh_terms: D000818:Animals; D017173:Caenorhabditis elegans / Q000378:metabolism; D004272:DNA, Mitochondrial / Q000235:genetics; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D008954:Models, Biological; D010085:Oxidative Phosphorylation; D056811:Unfolded Protein Response
chemical_list: D004272:DNA, Mitochondrial
keywords: ATFS-1; OXPHOS; UPR(mt); mitochondrial disease; mitochondrial heteroplasmy; mtDNA deletion