{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T02:28:08Z","timestamp":1791340088866,"version":"4.3.1"},"reference-count":44,"publisher":"Public Library of Science (PLoS)","issue":"10","license":[{"start":{"date-parts":[[2016,10,21]],"date-time":"2016-10-21T00:00:00Z","timestamp":1477008000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"The Scientific and Technological Research Council of Turkey (TUBITAK) 1001 Grant Project","award":["110T405"],"award-info":[{"award-number":["110T405"]}]},{"name":"The Scientific and Technological Research Council of Turkey (TUBITAK)","award":["BIDEB 2210"],"award-info":[{"award-number":["BIDEB 2210"]}]},{"name":"The Scientific and Technological Research Council of Turkey (TUBITAK)","award":["BIDEB 2210"],"award-info":[{"award-number":["BIDEB 2210"]}]},{"name":"The Scientific and Technological Research Council of Turkey (TUBITAK)","award":["TUBITAK 2216"],"award-info":[{"award-number":["TUBITAK 2216"]}]},{"name":"The Scientific and Technological Research Council of Turkey (TUBITAK)","award":["BIDEB 2210"],"award-info":[{"award-number":["BIDEB 2210"]}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0164864","type":"journal-article","created":{"date-parts":[[2016,10,21]],"date-time":"2016-10-21T16:43:53Z","timestamp":1477068233000},"page":"e0164864","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":35,"title":["IBMPFD Disease-Causing Mutant VCP\/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation"],"prefix":"10.1371","volume":"11","author":[{"given":"Oznur","family":"Bayraktar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ozlem","family":"Oral","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nur Mehpare","family":"Kocaturk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yunus","family":"Akkoc","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin","family":"Eberhart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali","family":"Kosar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Devrim","family":"Gozuacik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2016,10,21]]},"reference":[{"issue":"1\u20132","key":"ref1","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/j.jsb.2003.11.014","article-title":"Molecular perspectives on p97-VCP: progress in understanding its structure and diverse biological functions","volume":"146","author":"Q Wang","year":"2004","journal-title":"J Struct Biol"},{"issue":"7\u20138","key":"ref2","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1016\/S0960-8966(03)00070-1","article-title":"Clinical and genetic heterogeneity in chromosome 9p associated hereditary inclusion body myopathy: exclusion of GNE and three other candidate genes","volume":"13","author":"GD Watts","year":"2003","journal-title":"Neuromuscul Disord"},{"issue":"4","key":"ref3","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/ng1332","article-title":"Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein","volume":"36","author":"GD Watts","year":"2004","journal-title":"Nat Genet"},{"issue":"1","key":"ref4","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1002\/mus.20890","article-title":"An Italian family with inclusion-body myopathy and frontotemporal dementia due to mutation in the VCP gene","volume":"37","author":"T Gidaro","year":"2008","journal-title":"Muscle & nerve"},{"issue":"Pt 2","key":"ref5","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1093\/brain\/awl238","article-title":"Pathological consequences of VCP mutations on human striated muscle","volume":"130","author":"CU Hubbers","year":"2007","journal-title":"Brain"},{"issue":"4","key":"ref6","first-page":"374","article-title":"A Brazilian family with hereditary inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia","volume":"44","author":"RD Fanganiello","year":"2011","journal-title":"Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas \/ Sociedade Brasileira de Biofisica [et al]"},{"key":"ref7","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":"8","key":"ref8","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/j.nmd.2011.05.008","article-title":"Distinct distal myopathy phenotype caused by VCP gene mutation in a Finnish family","volume":"21","author":"J Palmio","year":"2011","journal-title":"Neuromuscul Disord"},{"issue":"3","key":"ref9","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1002\/ana.20407","article-title":"Mutant valosin-containing protein causes a novel type of frontotemporal dementia","volume":"57","author":"R Schroder","year":"2005","journal-title":"Ann Neurol"},{"issue":"12","key":"ref10","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1016\/j.bbadis.2008.09.003","article-title":"VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder","volume":"1782","author":"VE Kimonis","year":"2008","journal-title":"Biochim Biophys Acta"},{"issue":"10","key":"ref11","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1136\/jnnp.2007.131334","article-title":"TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia","volume":"79","author":"CC Weihl","year":"2008","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref12","article-title":"Regulation of Diacylglycerol Acyltransferase 2 (DGAT2) Protein Stability by gp78-Associated ER-associated degradation (ERAD)","author":"K Choi","year":"2014","journal-title":"FEBS J"},{"issue":"47","key":"ref13","doi-asserted-by":"crossref","first-page":"33861","DOI":"10.1074\/jbc.M113.492199","article-title":"Interaction between salt-inducible kinase 2 (SIK2) and p97\/valosin-containing protein (VCP) regulates endoplasmic reticulum (ER)-associated protein degradation in mammalian cells","volume":"288","author":"FC Yang","year":"2013","journal-title":"J Biol Chem"},{"issue":"12","key":"ref14","doi-asserted-by":"crossref","first-page":"2672","DOI":"10.1016\/j.bbapap.2013.09.021","article-title":"Structural and mechanistic insights into the arginine\/lysine-rich peptide motifs that interact with P97\/VCP","volume":"1834","author":"S Liu","year":"2013","journal-title":"Biochim Biophys Acta"},{"issue":"9","key":"ref15","doi-asserted-by":"crossref","first-page":"e74415","DOI":"10.1371\/journal.pone.0074415","article-title":"Combined inhibition of p97 and the proteasome causes lethal disruption of the secretory apparatus in multiple myeloma cells","volume":"8","author":"HW Auner","year":"2013","journal-title":"PLoS One"},{"issue":"12","key":"ref16","doi-asserted-by":"crossref","first-page":"1983","DOI":"10.1007\/s10529-013-1295-2","article-title":"BRSK2 is a valosin-containing protein (VCP)-interacting protein that affects VCP functioning in endoplasmic reticulum-associated degradation","volume":"35","author":"Y Wang","year":"2013","journal-title":"Biotechnol Lett"},{"issue":"4","key":"ref17","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1016\/j.biocel.2013.01.006","article-title":"A unique IBMPFD-related P97\/VCP mutation with differential binding pattern and subcellular localization","volume":"45","author":"Y Erzurumlu","year":"2013","journal-title":"Int J Biochem Cell Biol"},{"issue":"15","key":"ref18","doi-asserted-by":"crossref","first-page":"2921","DOI":"10.1016\/j.yexcr.2006.05.013","article-title":"Destabilization of the VCP-Ufd1-Npl4 complex is associated with decreased levels of ERAD substrates","volume":"312","author":"D Nowis","year":"2006","journal-title":"Exp Cell Res"},{"issue":"2","key":"ref19","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1093\/hmg\/ddi426","article-title":"Inclusion body myopathy-associated mutations in p97\/VCP impair endoplasmic reticulum-associated degradation","volume":"15","author":"CC Weihl","year":"2006","journal-title":"Hum Mol Genet"},{"issue":"1","key":"ref20","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/j.bbamcr.2013.03.031","article-title":"Create and preserve: proteostasis in development and aging is governed by Cdc48\/p97\/VCP","volume":"1843","author":"A Franz","year":"2014","journal-title":"Biochim Biophys Acta"},{"issue":"2","key":"ref21","doi-asserted-by":"crossref","first-page":"217","DOI":"10.4161\/auto.6.2.11014","article-title":"VCP\/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD","volume":"6","author":"E Tresse","year":"2010","journal-title":"Autophagy"},{"issue":"6","key":"ref22","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1083\/jcb.200908115","article-title":"Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease","volume":"187","author":"JS Ju","year":"2009","journal-title":"J Cell Biol"},{"issue":"22","key":"ref23","doi-asserted-by":"crossref","first-page":"2861","DOI":"10.1101\/gad.1599207","article-title":"Autophagy: process and function","volume":"21","author":"N Mizushima","year":"2007","journal-title":"Genes Dev"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/S0070-2153(06)78006-1","article-title":"Autophagy and cell death","volume":"78","author":"D Gozuacik","year":"2007","journal-title":"Curr Top Dev Biol"},{"issue":"7095","key":"ref25","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1038\/nature04724","article-title":"Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice","volume":"441","author":"T Hara","year":"2006","journal-title":"Nature"},{"issue":"21","key":"ref26","doi-asserted-by":"crossref","first-page":"5720","DOI":"10.1093\/emboj\/19.21.5720","article-title":"LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing","volume":"19","author":"Y Kabeya","year":"2000","journal-title":"EMBO J"},{"issue":"12","key":"ref27","doi-asserted-by":"crossref","first-page":"2503","DOI":"10.1016\/j.biocel.2004.05.009","article-title":"LC3 conjugation system in mammalian autophagy","volume":"36","author":"I Tanida","year":"2004","journal-title":"Int J Biochem Cell Biol"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"16","DOI":"10.3389\/fncel.2015.00016","article-title":"Mitochondrial function in neuronal cells depends on p97\/VCP\/Cdc48-mediated quality control","volume":"9","author":"L Fang","year":"2015","journal-title":"Front Cell Neurosci"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.mito.2015.02.004","article-title":"In vitro studies in VCP-associated multisystem proteinopathy suggest altered mitochondrial bioenergetics","volume":"22","author":"A Nalbandian","year":"2015","journal-title":"Mitochondrion"},{"key":"ref30","article-title":"Visualization of retroviral replication in living cells reveals budding into multivesicular bodies","author":"NM Sherer","year":"2003","journal-title":"Visualization of retroviral replication in living cells reveals budding into multivesicular bodies"},{"issue":"1","key":"ref31","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1083\/jcb.201009067","article-title":"p62 Targeting to the autophagosome formation site requires self-oligomerization but not LC3 binding","volume":"192","author":"E Itakura","year":"2011","journal-title":"J Cell Biol"},{"issue":"Unit13 4","key":"ref32","doi-asserted-by":"crossref","DOI":"10.1002\/0471142905.hg1304s31","article-title":"Gene delivery to muscle","volume":"Chapter 13","author":"ML Springer","year":"2002","journal-title":"Current protocols in human genetics \/ editorial board, Jonathan L Haines [et al]"},{"issue":"8","key":"ref33","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1007\/s10495-012-0735-0","article-title":"Cleavage of Atg3 protein by caspase-8 regulates autophagy during receptor-activated cell death","volume":"17","author":"O Oral","year":"2012","journal-title":"Apoptosis"},{"key":"ref34","first-page":"36","article-title":"Image Processing with ImageJ","volume":"11","author":"MD Abramoff","year":"2004","journal-title":"Biophotonic International"},{"issue":"3","key":"ref35","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1002\/mus.21225","article-title":"A novel mutation in the VCP gene (G157R) in a German family with inclusion-body myopathy with Paget disease of bone and frontotemporal dementia","volume":"39","author":"A Djamshidian","year":"2009","journal-title":"Muscle & nerve"},{"issue":"5","key":"ref36","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/j.nmd.2009.02.012","article-title":"Clinical outcome in 19 French and Spanish patients with valosin-containing protein myopathy associated with Paget's disease of bone and frontotemporal dementia","volume":"19","author":"T Stojkovic","year":"2009","journal-title":"Neuromuscul Disord"},{"issue":"6","key":"ref37","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1093\/hmg\/dds524","article-title":"mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy","volume":"22","author":"JK Ching","year":"2013","journal-title":"Hum Mol Genet"},{"issue":"10","key":"ref38","doi-asserted-by":"crossref","first-page":"e46879","DOI":"10.1371\/journal.pone.0046879","article-title":"Heteromeric p97\/p97R155C complexes induce dominant negative changes in wild-type and autophagy 9-deficient Dictyostelium strains","volume":"7","author":"K Arhzaouy","year":"2012","journal-title":"PLoS One"},{"issue":"12","key":"ref39","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1111\/gtc.12103","article-title":"Different dynamic movements of wild-type and pathogenic VCPs and their cofactors to damaged mitochondria in a Parkin-mediated mitochondrial quality control system","volume":"18","author":"Y Kimura","year":"2013","journal-title":"Genes Cells"},{"issue":"1","key":"ref40","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.neuron.2013.02.029","article-title":"VCP is essential for mitochondrial quality control by PINK1\/Parkin and this function is impaired by VCP mutations","volume":"78","author":"NC Kim","year":"2013","journal-title":"Neuron"},{"issue":"10","key":"ref41","doi-asserted-by":"crossref","first-page":"977","DOI":"10.1038\/sj.cdd.4400907","article-title":"VCP\/p97 in abnormal protein aggregates, cytoplasmic vacuoles, and cell death, phenotypes relevant to neurodegeneration","volume":"8","author":"M Hirabayashi","year":"2001","journal-title":"Cell Death Differ"},{"issue":"4","key":"ref42","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1046\/j.1440-1789.2003.00514.x","article-title":"Two distinct subgroups of senile dementia of Alzheimer type: quantitative study of neurofibrillary tangles","volume":"23","author":"Y Mizuno","year":"2003","journal-title":"Neuropathology"},{"issue":"1","key":"ref43","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.yexmp.2004.08.006","article-title":"RNA interference of VCP\/p97 increases Mallory body formation","volume":"78","author":"L Nan","year":"2005","journal-title":"Exp Mol Pathol"},{"issue":"5","key":"ref44","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.neuron.2010.11.036","article-title":"Exome sequencing reveals VCP mutations as a cause of familial ALS","volume":"68","author":"JO Johnson","year":"2010","journal-title":"Neuron"}],"updated-by":[{"DOI":"10.1371\/journal.pone.0167192","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2016,11,18]],"date-time":"2016-11-18T00:00:00Z","timestamp":1479427200000}}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0164864","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,10,20]],"date-time":"2018-10-20T07:06:15Z","timestamp":1540019175000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0164864"}},"subtitle":[],"editor":[{"given":"Srinivasa M","family":"Srinivasula","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2016,10,21]]},"references-count":44,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2016,10,21]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0164864","relation":{"correction":[{"id-type":"doi","id":"10.1371\/journal.pone.0167192","asserted-by":"object"}]},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,10,21]]}}}