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Sci"},{"key":"ref77","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1038\/nature12188","article-title":"Mitonuclear protein imbalance as a conserved longevity mechanism","volume":"497","author":"RH Houtkooper","year":"2013","journal-title":"Nature"},{"key":"ref78","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1038\/nature21695","article-title":"Cytosolic proteostasis through importing of misfolded proteins into mitochondria","volume":"543","author":"LH Ruan","year":"2017","journal-title":"Nature"},{"key":"ref79","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.cub.2016.10.044","article-title":"The Mitochondrial Lon Protease Is Required for Age-Specific and Sex-Specific Adaptation to Oxidative Stress","volume":"27","author":"LCD Pomatto","year":"2017","journal-title":"Current Biology"},{"key":"ref80","doi-asserted-by":"crossref","first-page":"7607","DOI":"10.1073\/pnas.0900688106","article-title":"Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity","volume":"106","author":"YJ Zhang","year":"2009","journal-title":"Proceedings Of the National Academy Of Sciences Of the United States Of America"},{"key":"ref81","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1159\/000287952","article-title":"Caspase-Cleaved Transactivation Response DNA-Binding Protein 43 in Parkinson's Disease and Dementia with Lewy Bodies","volume":"7","author":"P Kokoulina","year":"2010","journal-title":"Neurodegenerative Diseases"},{"key":"ref82","doi-asserted-by":"crossref","first-page":"2455","DOI":"10.1002\/pmic.201200006","article-title":"Asparaginyl endopeptidase cleaves TDP-43 in brain","volume":"12","author":"JH Herskowitz","year":"2012","journal-title":"Proteomics"},{"key":"ref83","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.1038\/jcbfm.2014.105","article-title":"Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injury","volume":"34","author":"ZH Yang","year":"2014","journal-title":"Journal Of Cerebral Blood Flow And Metabolism"},{"key":"ref84","doi-asserted-by":"crossref","first-page":"6183","DOI":"10.1038\/ncomms7183","article-title":"The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity","volume":"6","author":"Q Li","year":"2015","journal-title":"Nature Communications"},{"key":"ref85","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1038\/ncomms2303","article-title":"A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology","volume":"3","author":"T Yamashita","year":"2012","journal-title":"Nature Communications"},{"key":"ref86","doi-asserted-by":"crossref","first-page":"e0177181","DOI":"10.1371\/journal.pone.0177181","article-title":"Truncation of the TAR DNA-binding protein 43 is not a prerequisite for cytoplasmic relocalization, and is suppressed by caspase inhibition and by introduction of the A90V sequence variant","volume":"12","author":"HJ Wobst","year":"2017","journal-title":"Plos One"},{"key":"ref87","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1007\/s13238-011-1065-7","article-title":"Expression of human FUS protein in Drosophila leads to progressive neurodegeneration","volume":"2","author":"Y Chen","year":"2011","journal-title":"Protein Cell"},{"key":"ref88","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1038\/nature721","article-title":"Crumbs, the Drosophila homologue of human CRB1\/RP12, is essential for photoreceptor morphogenesis","volume":"416","author":"M Pellikka","year":"2002","journal-title":"Nature"},{"key":"ref89","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.molcel.2015.11.017","article-title":"Mitoguardin Regulates Mitochondrial Fusion through MitoPLD and Is Required for Neuronal Homeostasis","volume":"61","author":"YP Zhang","year":"2016","journal-title":"Molecular Cell"},{"key":"ref90","first-page":"53149","article-title":"Preparation of Mitochondrial 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