title: Integrating the UPRmtinto the mitochondrial maintenance network.
abstract: Mitochondrial function is central to many different processes in the cell, from oxidative phosphorylation to the synthesis of iron-sulfur clusters. Therefore, mitochondrial dysfunction underlies a diverse array of diseases, from neurodegenerative diseases to cancer. Stress can be communicated to the cytosol and nucleus from the mitochondria through many different signals, and in response the cell can effect everything from transcriptional to post-transcriptional responses to protect the mitochondrial network. How these responses are coordinated have only recently begun to be understood. In this review, we explore how the cell maintains mitochondrial function, focusing on the mitochondrial unfolded protein response (UPRmt), a transcriptional response that can activate a wide array of programs to repair and restore mitochondrial function.
authors.affiliation: a Department of Molecular Cell and Cancer Biology , University of Massachusetts Medical School , Worcester , MA , USA.|b BCMB Allied Program , Weill Cornell Medical College , New York , NY , USA.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Fiorese
authors.affiliation: a Department of Molecular Cell and Cancer Biology , University of Massachusetts Medical School , Worcester , MA , USA.|b BCMB Allied Program , Weill Cornell Medical College , New York , NY , USA.
authors.forename: Cole M
authors.initials: CM
authors.lastname: Haynes
mesh_terms: D000818:Animals; D006801:Humans; D008928:Mitochondria / Q000378:metabolism* / Q000473:pathology; D009369:Neoplasms / Q000378:metabolism* / Q000473:pathology; D019636:Neurodegenerative Diseases / Q000378:metabolism* / Q000473:pathology; D056811:Unfolded Protein Response
keywords: Mitochondrial unfolded protein response; integrated stress response; mitochondria quality control; mitochondrial dysfunction; mitochondrial stress response