{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T17:11:53Z","timestamp":1790701913232,"version":"4.1.0"},"reference-count":62,"publisher":"Public Library of Science (PLoS)","issue":"9","license":[{"start":{"date-parts":[[2015,9,2]],"date-time":"2015-09-02T00:00:00Z","timestamp":1441152000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0137009","type":"journal-article","created":{"date-parts":[[2015,9,2]],"date-time":"2015-09-02T14:26:55Z","timestamp":1441204015000},"page":"e0137009","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":33,"title":["Antioxidant Treatment and Induction of Autophagy Cooperate to Reduce Desmin Aggregation in a Cellular Model of Desminopathy"],"prefix":"10.1371","volume":"10","author":[{"given":"Eva","family":"Cabet","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Batonnet-Pichon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Delort","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Blandine","family":"Gausser\u00e8s","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Vicart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Lilienbaum","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2015,9,2]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1146\/annurev.genom.7.080505.115737","article-title":"Protein misfolding and human disease","volume":"7","author":"N Gregersen","year":"2006","journal-title":"Annu Rev Genomics Hum Genet"},{"issue":"8","key":"ref2","doi-asserted-by":"crossref","first-page":"a004374","DOI":"10.1101\/cshperspect.a004374","article-title":"Cellular strategies of protein quality control","volume":"3","author":"B Chen","year":"2011","journal-title":"Cold Spring Harb Perspect Biol"},{"issue":"5","key":"ref3","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1093\/jb\/mvp139","article-title":"Quality control against misfolded proteins in the cytosol: a network for cell survival","volume":"146","author":"H Kubota","year":"2009","journal-title":"J Biochem"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1543","DOI":"10.1100\/tsw.2010.152","article-title":"Heat shock proteins: cell protection through protein triage","volume":"10","author":"D Lanneau","year":"2010","journal-title":"ScientificWorldJournal"},{"issue":"5","key":"ref5","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1111\/j.1600-0854.2012.01330.x","article-title":"Quality control compartments coming of age","volume":"13","author":"T Ben-Gedalya","year":"2012","journal-title":"Traffic"},{"issue":"5","key":"ref6","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1002\/iub.189","article-title":"Oxidized proteins: mechanisms of removal and consequences of accumulation","volume":"61","author":"RA Dunlop","year":"2009","journal-title":"IUBMB Life"},{"issue":"1","key":"ref7","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1007\/s00401-012-1057-6","article-title":"Desminopathies: pathology and mechanisms","volume":"125","author":"CS Clemen","year":"2013","journal-title":"Acta Neuropathol"},{"issue":"5","key":"ref8","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1097\/WCO.0b013e328364d6b1","article-title":"Myofibrillar myopathies: new developments","volume":"26","author":"M Olive","year":"2013","journal-title":"Curr Opin Neurol"},{"issue":"3","key":"ref9","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/j.nmd.2010.12.007","article-title":"Myofibrillar myopathies","volume":"21","author":"D Selcen","year":"2011","journal-title":"Neuromuscul Disord"},{"issue":"4","key":"ref10","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1111\/j.1399-0004.2010.01512.x","article-title":"Desmin-related myopathy","volume":"80","author":"KY van Spaendonck-Zwarts","year":"2011","journal-title":"Clin Genet"},{"issue":"1","key":"ref11","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00401-012-1071-8","article-title":"Protein aggregate myopathies: the many faces of an expanding disease group","volume":"125","author":"R Schr\u00f6der","year":"2013","journal-title":"Acta Neuropathol"},{"issue":"3","key":"ref12","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1007\/BF00227598","article-title":"Effect of anabolic steroids on rat heart muscle cells. I. Intermediate filaments","volume":"180","author":"H Behrendt","year":"1977","journal-title":"Cell Tissue Res"},{"issue":"3","key":"ref13","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/0022-2828(73)90065-5","article-title":"Intermyofibrillar and nuclear-myofibrillar connections in human and canine myocardium. An ultrastructural study","volume":"5","author":"VJ Ferrans","year":"1973","journal-title":"J Mol Cell Cardiol"},{"issue":"12","key":"ref14","doi-asserted-by":"crossref","first-page":"4344","DOI":"10.1073\/pnas.73.12.4344","article-title":"Immunological characterization of the subunit of the 100 A filaments from muscle cells","volume":"73","author":"E Lazarides","year":"1976","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"5","key":"ref15","doi-asserted-by":"crossref","first-page":"2993","DOI":"10.1016\/S0006-3495(04)74349-0","article-title":"Structural and functional roles of desmin in mouse skeletal muscle during passive deformation","volume":"86","author":"SB Shah","year":"2004","journal-title":"Biophys J"},{"key":"ref16","first-page":"704061","article-title":"Influences of desmin and keratin 19 on passive biomechanical properties of mouse skeletal muscle","volume":"2012","author":"SB Shah","year":"2012","journal-title":"J Biomed Biotechnol"},{"issue":"42","key":"ref17","doi-asserted-by":"crossref","first-page":"15099","DOI":"10.1073\/pnas.0504568102","article-title":"Severe muscle disease-causing desmin mutations interfere with in vitro filament assembly at distinct stages","volume":"102","author":"H B\u00e4r","year":"2005","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"5","key":"ref18","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1016\/j.jmb.2006.05.068","article-title":"Impact of disease mutations on the desmin filament assembly process","volume":"360","author":"H B\u00e4r","year":"2006","journal-title":"J Mol Biol"},{"issue":"9","key":"ref19","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1002\/humu.20351","article-title":"Variable pathogenic potentials of mutations located in the desmin alpha-helical domain","volume":"27","author":"B Goudeau","year":"2006","journal-title":"Hum Mutat"},{"issue":"8","key":"ref20","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/S1050-1738(02)00184-6","article-title":"Desmin cytoskeleton: a potential regulator of muscle mitochondrial behavior and function","volume":"12","author":"Y Capetanaki","year":"2002","journal-title":"Trends Cardiovasc Med"},{"issue":"1","key":"ref21","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1046\/j.1365-2990.2003.00428.x","article-title":"Mitochondrial dysfunction in myofibrillar myopathy","volume":"29","author":"J Reimann","year":"2003","journal-title":"Neuropathol Appl Neurobiol"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"1299","DOI":"10.1016\/B978-0-444-59565-2.00002-2","article-title":"Main steps of skeletal muscle development in the human: morphological analysis and ultrastructural characteristics of developing human muscle","volume":"113","author":"NB Romero","year":"2013","journal-title":"Handb Clin Neurol"},{"key":"ref23","author":"A Lilienbaum"},{"issue":"2","key":"ref24","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1093\/cvr\/cvp287","article-title":"The ubiquitin-proteasome system in cardiac proteinopathy: a quality control perspective","volume":"85","author":"H Su","year":"2010","journal-title":"Cardiovasc Res"},{"issue":"3","key":"ref25","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1007\/s10522-013-9429-4","article-title":"Age-dependent alteration in muscle regeneration: the critical role of tissue niche","volume":"14","author":"L Barberi","year":"2013","journal-title":"Biogerontology"},{"issue":"5","key":"ref26","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1002\/humu.1210","article-title":"Structural and functional analysis of a new desmin variant causing desmin-related myopathy","volume":"18","author":"B Goudeau","year":"2001","journal-title":"Hum Mutat"},{"issue":"6","key":"ref27","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1016\/0092-8674(93)90635-4","article-title":"Seeding &quot;one-dimensional crystallization&quot; of amyloid: a pathogenic mechanism in Alzheimer&apos;s disease and scrapie?","volume":"73","author":"JT Jarrett","year":"1993","journal-title":"Cell"},{"issue":"18","key":"ref28","doi-asserted-by":"crossref","first-page":"3570","DOI":"10.1016\/j.yexcr.2006.07.025","article-title":"Cell signaling pathways to alphaB-crystallin following stresses of the cytoskeleton","volume":"312","author":"N Launay","year":"2006","journal-title":"Exp Cell Res"},{"issue":"11","key":"ref29","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1002\/cm.21071","article-title":"Rho GTPases in animal cell cytokinesis: an occupation by the one percent","volume":"69","author":"SN Jordan","year":"2012","journal-title":"Cytoskeleton (Hoboken)"},{"issue":"2","key":"ref30","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1042\/BC20070109","article-title":"A tale of two Paks","volume":"100","author":"LE Arias-Romero","year":"2008","journal-title":"Biol Cell"},{"issue":"2","key":"ref31","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1038\/nrm2847","article-title":"PKC and the control of localized signal dynamics","volume":"11","author":"C Rosse","year":"2010","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"1\u20132","key":"ref32","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1007\/s11010-010-0563-x","article-title":"Metals, oxidative stress and neurodegenerative disorders","volume":"345","author":"K Jomova","year":"2010","journal-title":"Mol Cell Biochem"},{"issue":"10","key":"ref33","doi-asserted-by":"crossref","first-page":"e76361","DOI":"10.1371\/journal.pone.0076361","article-title":"N-acetyl-L-cysteine prevents stress-induced desmin aggregation in cellular models of desminopathy","volume":"8","author":"BD Segard","year":"2013","journal-title":"PLoS One"},{"issue":"5","key":"ref34","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1038\/nchembio.79","article-title":"Novel targets for Huntington&apos;s disease in an mTOR-independent autophagy pathway","volume":"4","author":"A Williams","year":"2008","journal-title":"Nat Chem Biol"},{"issue":"3","key":"ref35","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/j.cell.2010.01.028","article-title":"Methods in mammalian autophagy research","volume":"140","author":"N Mizushima","year":"2010","journal-title":"Cell"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0076-6879(08)03601-X","article-title":"Monitoring autophagy in mammalian cultured cells through the dynamics of LC3","volume":"452","author":"S Kimura","year":"2009","journal-title":"Methods Enzymol"},{"issue":"1","key":"ref37","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.molcel.2011.03.017","article-title":"Rac1 regulates the activity of mTORC1 and mTORC2 and controls cellular size","volume":"42","author":"A Saci","year":"2011","journal-title":"Mol Cell"},{"issue":"4","key":"ref38","first-page":"251","article-title":"Increased autophagocytosis induced by colchicine in rat sympathetic neurons","volume":"30","author":"PG Nicolescu","year":"1984","journal-title":"Morphol Embryol (Bucur)"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1111\/j.1749-6632.1998.tb09908.x","article-title":"Membrane and receptor modifications of oxidative stress vulnerability in aging. Nutritional considerations","volume":"854","author":"JA Joseph","year":"1998","journal-title":"Ann N Y Acad Sci"},{"issue":"7","key":"ref40","doi-asserted-by":"crossref","first-page":"1806","DOI":"10.1172\/JCI38027","article-title":"Tragedy in a heartbeat: malfunctioning desmin causes skeletal and cardiac muscle disease","volume":"119","author":"LG Goldfarb","year":"2009","journal-title":"J Clin Invest"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"1337","DOI":"10.1016\/B978-0-444-59565-2.00005-8","article-title":"Myofibrillar myopathies","volume":"113","author":"KG Claeys","year":"2013","journal-title":"Handb Clin Neurol"},{"issue":"6","key":"ref42","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1159\/000350111","article-title":"Enhancement of autophagy by simvastatin through inhibition of Rac1-mTOR signaling pathway in coronary arterial myocytes","volume":"31","author":"YM Wei","year":"2013","journal-title":"Cell Physiol Biochem"},{"issue":"1","key":"ref43","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00418-013-1148-z","article-title":"Posttranslational modifications of desmin and their implication in biological processes and pathologies","volume":"141","author":"DL Winter","year":"2014","journal-title":"Histochem Cell Biol"},{"issue":"2","key":"ref44","doi-asserted-by":"crossref","first-page":"78","DOI":"10.4161\/cl.19817","article-title":"Untangling the complexity of PAK1 dynamics: The future challenge","volume":"2","author":"MC Parrini","year":"2012","journal-title":"Cell Logist"},{"issue":"6458","key":"ref45","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1038\/367040a0","article-title":"A brain serine\/threonine protein kinase activated by Cdc42 and Rac1","volume":"367","author":"E Manser","year":"1994","journal-title":"Nature"},{"issue":"1","key":"ref46","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/S1097-2765(01)00428-2","article-title":"Pak1 kinase homodimers are autoinhibited in trans and dissociated upon activation by Cdc42 and Rac1","volume":"9","author":"MC Parrini","year":"2002","journal-title":"Mol Cell"},{"issue":"16","key":"ref47","doi-asserted-by":"crossref","first-page":"11836","DOI":"10.1074\/jbc.M610106200","article-title":"p21-activated kinase 1 is activated through the mammalian target of rapamycin\/p70 S6 kinase pathway and regulates the replication of hepatitis C virus in human hepatoma cells","volume":"282","author":"H Ishida","year":"2007","journal-title":"J Biol Chem"},{"issue":"5","key":"ref48","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1096\/fj.12-215475","article-title":"Intracellular signaling in ER stress-induced autophagy in skeletal muscle cells","volume":"27","author":"L Madaro","year":"2013","journal-title":"FASEB J"},{"issue":"2","key":"ref49","doi-asserted-by":"crossref","first-page":"H570","DOI":"10.1152\/ajpheart.00716.2009","article-title":"Autophagy and protein kinase C are required for cardioprotection by sulfaphenazole","volume":"298","author":"C Huang","year":"2010","journal-title":"Am J Physiol Heart Circ Physiol"},{"issue":"2","key":"ref50","doi-asserted-by":"crossref","first-page":"244","DOI":"10.4161\/auto.5.2.7549","article-title":"PKC delta signaling: a dual role in regulating hypoxic stress-induced autophagy and apoptosis","volume":"5","author":"JL Chen","year":"2009","journal-title":"Autophagy"},{"issue":"4","key":"ref51","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1111\/j.1750-3639.2007.00087.x","article-title":"Oxidative stress in desminopathies and myotilinopathies: a link between oxidative damage and abnormal protein aggregation","volume":"17","author":"A Janu\u00e9","year":"2007","journal-title":"Brain Pathol"},{"issue":"8","key":"ref52","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1097\/nen.0b013e3181256b4c","article-title":"Desmin is oxidized and nitrated in affected muscles in myotilinopathies and desminopathies","volume":"66","author":"A Janu\u00e9","year":"2007","journal-title":"J Neuropathol Exp Neurol"},{"issue":"41","key":"ref53","doi-asserted-by":"crossref","first-page":"34764","DOI":"10.1074\/jbc.M112.387035","article-title":"Post-aggregation oxidation of mutant huntingtin controls the interactions between aggregates","volume":"287","author":"Y Mitomi","year":"2012","journal-title":"J Biol Chem"},{"issue":"8","key":"ref54","doi-asserted-by":"crossref","first-page":"3121","DOI":"10.1021\/jm3015926","article-title":"Target- and mechanism-based therapeutics for neurodegenerative diseases: strength in numbers","volume":"56","author":"PC Trippier","year":"2013","journal-title":"J Med Chem"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/j.redox.2013.12.013","article-title":"Autophagy as an essential cellular antioxidant pathway in neurodegenerative disease","volume":"2","author":"S Giordano","year":"2014","journal-title":"Redox Biol"},{"issue":"28","key":"ref56","doi-asserted-by":"crossref","first-page":"9745","DOI":"10.1073\/pnas.0706802105","article-title":"Autophagy is an adaptive response in desmin-related cardiomyopathy","volume":"105","author":"P Tannous","year":"2008","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"11","key":"ref57","doi-asserted-by":"crossref","first-page":"1540","DOI":"10.1093\/hmg\/ddn042","article-title":"Autophagy induced by Alexander disease-mutant GFAP accumulation is regulated by p38\/MAPK and mTOR signaling pathways","volume":"17","author":"G Tang","year":"2008","journal-title":"Hum Mol Genet"},{"issue":"144","key":"ref58","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.3003802","article-title":"Rapamycin reverses elevated mTORC1 signaling in lamin A\/C-deficient mice, rescues cardiac and skeletal muscle function, and extends survival","volume":"4","author":"FJ Ramos","year":"2012","journal-title":"Sci Transl Med"},{"issue":"4","key":"ref59","doi-asserted-by":"crossref","first-page":"429","DOI":"10.4161\/auto.7.4.14393","article-title":"The Parkinson disease protein alpha-synuclein inhibits autophagy","volume":"7","author":"AR Winslow","year":"2011","journal-title":"Autophagy"},{"issue":"16","key":"ref60","doi-asserted-by":"crossref","first-page":"2570","DOI":"10.1093\/hmg\/ddn157","article-title":"Autophagy-mediated clearance of aggresomes is not a universal phenomenon","volume":"17","author":"ES Wong","year":"2008","journal-title":"Hum Mol Genet"},{"issue":"3","key":"ref61","doi-asserted-by":"crossref","first-page":"422","DOI":"10.4161\/auto.22921","article-title":"Impairment of protein degradation in myofibrillar myopathy caused by FLNC\/filamin C mutations","volume":"9","author":"RA Kley","year":"2013","journal-title":"Autophagy"},{"issue":"2","key":"ref62","doi-asserted-by":"crossref","first-page":"151","DOI":"10.4161\/auto.5338","article-title":"Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes","volume":"4","author":"DJ Klionsky","year":"2008","journal-title":"Autophagy"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0137009","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,10,23]],"date-time":"2018-10-23T00:46:02Z","timestamp":1540255562000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0137009"}},"subtitle":[],"editor":[{"given":"Gerhard","family":"Wiche","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2015,9,2]]},"references-count":62,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2015,9,2]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0137009","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,9,2]]}}}