{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T03:15:50Z","timestamp":1785554150011,"version":"3.56.0"},"reference-count":49,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2019,6,1]],"date-time":"2019-06-01T00:00:00Z","timestamp":1559347200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,6,1]],"date-time":"2019-06-01T00:00:00Z","timestamp":1559347200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,6,1]],"date-time":"2020-06-01T00:00:00Z","timestamp":1590969600000},"content-version":"vor","delay-in-days":366,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"Chinese National Natural Science Foundation Projects","doi-asserted-by":"crossref","award":["31471326"],"award-info":[{"award-number":["31471326"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Jiangsu Specially Appointed Professor program"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 NS088192"],"award-info":[{"award-number":["R01 NS088192"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"published-print":{"date-parts":[[2019,6]]},"DOI":"10.1016\/j.bbadis.2019.02.015","type":"journal-article","created":{"date-parts":[[2019,2,23]],"date-time":"2019-02-23T10:15:23Z","timestamp":1550916923000},"page":"1428-1435","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":40,"title":["Mutant huntingtin inhibits the mitochondrial unfolded protein response by impairing ABCB10 mRNA stability"],"prefix":"10.1016","volume":"1865","author":[{"given":"Zixing","family":"Fu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fang","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chunyue","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beifang","family":"Jin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yueqing","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mingliang","family":"Tang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xin","family":"Qi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xing","family":"Guo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbadis.2019.02.015_bb0005","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1038\/sj.embor.7400250","article-title":"Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series","volume":"5","author":"Landles","year":"2004","journal-title":"EMBO Rep."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0010","doi-asserted-by":"crossref","first-page":"1853","DOI":"10.1038\/emboj.2012.65","article-title":"Shaping the role of mitochondria in the pathogenesis of Huntington's disease","volume":"31","author":"Costa","year":"2012","journal-title":"EMBO J."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0015","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0020","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1016\/j.tins.2008.09.004","article-title":"Mutant huntingtin and mitochondrial dysfunction","volume":"31","author":"Bossy-Wetzel","year":"2008","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0025","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1002\/9780470725207.ch5","article-title":"Transcriptional control of mitochondrial energy metabolism through the PGC1 coactivators","volume":"287","author":"Spiegelman","year":"2007","journal-title":"Novartis Found. Symp."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0030","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.cell.2006.09.015","article-title":"Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration","volume":"127","author":"Cui","year":"2006","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0035","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/nm.2313","article-title":"Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity","volume":"17","author":"Song","year":"2011","journal-title":"Nat. Med."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0040","doi-asserted-by":"crossref","first-page":"5371","DOI":"10.1172\/JCI70911","article-title":"Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration","volume":"123","author":"Guo","year":"2013","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0045","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1016\/j.bbadis.2016.11.026","article-title":"VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease","volume":"1863","author":"Guo","year":"2017","journal-title":"Biochim. Biophys. Acta Mol. Basis Dis."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0050","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms12646","article-title":"VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease","volume":"7","author":"Guo","year":"2016","journal-title":"Nat. Commun."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0055","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.ceb.2014.12.003","article-title":"The mitochondrial unfolded protein response-synchronizing genomes","volume":"33","author":"Jovaisaite","year":"2015","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0060","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1242\/jeb.090738","article-title":"The mitochondrial unfolded protein response, a conserved stress response pathway with implications in health and disease","volume":"217","author":"Jovaisaite","year":"2014","journal-title":"J. Exp. Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0065","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/j.molcel.2010.01.015","article-title":"The matrix peptide exporter HAF-1 signals a mitochondrial UPR by activating the transcription factor ZC376.7 in C. elegans","volume":"37","author":"Haynes","year":"2010","journal-title":"Mol. Cell"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0070","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1016\/j.devcel.2007.07.016","article-title":"ClpP mediates activation of a mitochondrial unfolded protein response in C. elegans","volume":"13","author":"Haynes","year":"2007","journal-title":"Dev. Cell"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0075","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1126\/science.1223560","article-title":"Mitochondrial import efficiency of ATFS-1 regulates mitochondrial UPR activation","volume":"337","author":"Nargund","year":"2012","journal-title":"Science"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0080","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/ng.544","article-title":"Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28","volume":"42","author":"Di Bella","year":"2010","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0085","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1016\/S0092-8674(00)81203-9","article-title":"Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease","volume":"93","author":"Casari","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0090","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1083\/jcb.201110131","article-title":"The impact of the unfolded protein response on human disease","volume":"197","author":"Wang","year":"2012","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0095","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1016\/j.tcb.2014.05.003","article-title":"Proteostasis impairment in protein-misfolding and -aggregation diseases","volume":"24","author":"Hipp","year":"2014","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0100","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1101\/sqb.2011.76.010629","article-title":"Sirtuins, aging, and metabolism","volume":"76","author":"Guarente","year":"2011","journal-title":"Cold Spring Harb. Symp. Quant. Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0105","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/j.bbrc.2017.03.063","article-title":"ABCB10 depletion reduces unfolded protein response in mitochondria","volume":"486","author":"Yano","year":"2017","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0110","doi-asserted-by":"crossref","first-page":"4411","DOI":"10.1093\/emboj\/cdf445","article-title":"A mitochondrial specific stress response in mammalian cells","volume":"21","author":"Zhao","year":"2002","journal-title":"EMBO J."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0115","doi-asserted-by":"crossref","first-page":"e835","DOI":"10.1371\/journal.pone.0000835","article-title":"The chop gene contains an element for the positive regulation of the mitochondrial unfolded protein response","volume":"2","author":"Horibe","year":"2007","journal-title":"PLoS One"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0120","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1083\/jcb.201503107","article-title":"The unpredictability of prolonged activation of stress response pathways","volume":"209","author":"Lamech","year":"2015","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0125","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1161\/CIRCULATIONAHA.110.003418","article-title":"Mitochondrial transporter ATP binding cassette mitochondrial erythroid is a novel gene required for cardiac recovery after ischemia\/reperfusion","volume":"124","author":"Liesa","year":"2011","journal-title":"Circulation"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0130","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1136\/gutjnl-2011-300767","article-title":"Induction of dsRNA-activated protein kinase links mitochondrial unfolded protein response to the pathogenesis of intestinal inflammation","volume":"61","author":"Rath","year":"2012","journal-title":"Gut"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0135","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1128\/MCB.01337-13","article-title":"SirT3 regulates the mitochondrial unfolded protein response","volume":"34","author":"Papa","year":"2014","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0140","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1038\/cdd.2009.22","article-title":"Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway","volume":"16","author":"Liot","year":"2009","journal-title":"Cell Death Differ."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0145","doi-asserted-by":"crossref","first-page":"2423","DOI":"10.1126\/science.1056784","article-title":"Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity","volume":"291","author":"Nucifora","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0150","doi-asserted-by":"crossref","first-page":"6763","DOI":"10.1073\/pnas.100110097","article-title":"The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription","volume":"97","author":"Steffan","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0155","doi-asserted-by":"crossref","first-page":"2197","DOI":"10.1093\/hmg\/9.14.2197","article-title":"CREB-binding protein sequestration by expanded polyglutamine","volume":"9","author":"McCampbell","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0160","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1023\/B:SCAM.0000007124.19463.e5","article-title":"Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins","volume":"24","author":"Huang","year":"1998","journal-title":"Somat. Cell Mol. Genet."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0165","doi-asserted-by":"crossref","first-page":"1647","DOI":"10.1093\/hmg\/8.9.1647","article-title":"Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin","volume":"8","author":"Boutell","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0170","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1038\/ng1219","article-title":"Huntingtin interacts with REST\/NRSF to modulate the transcription of NRSE-controlled neuronal genes","volume":"35","author":"Zuccato","year":"2003","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0175","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1016\/0005-2787(75)90008-8","article-title":"Gene activation in human diploid cells. Age-dependent modifications in the stability of messenger RNAs for nonhistone chromosomal proteins","volume":"390","author":"Stein","year":"1975","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0180","doi-asserted-by":"crossref","first-page":"587","DOI":"10.2174\/1566524018666180308110130","article-title":"The mitochondrial unfolded protein response: role in cellular homeostasis and disease","volume":"17","author":"De","year":"2017","journal-title":"Curr. Mol. Med."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0185","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1038\/nrm.2017.110","article-title":"The mitochondrial UPR: mechanisms, physiological functions and implications in ageing","volume":"19","author":"Shpilka","year":"2018","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0190","doi-asserted-by":"crossref","first-page":"a008888","DOI":"10.1101\/cshperspect.a008888","article-title":"Genetics of Parkinson's disease","volume":"2","author":"Klein","year":"2012","journal-title":"Cold Spring Harb. Perspect. Med."},{"issue":"2","key":"10.1016\/j.bbadis.2019.02.015_bb0195","doi-asserted-by":"crossref","first-page":"R115","DOI":"10.1093\/hmg\/ddm213","article-title":"Polyglutamine diseases: emerging concepts in pathogenesis and therapy","volume":"16","author":"Shao","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0200","doi-asserted-by":"crossref","first-page":"45004","DOI":"10.1074\/jbc.M205259200","article-title":"Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities","volume":"277","author":"Li","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0205","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.neuron.2017.03.027","article-title":"Polyglutamine-expanded huntingtin exacerbates age-related disruption of nuclear integrity and nucleocytoplasmic transport","volume":"94","author":"Gasset-Rosa","year":"2017","journal-title":"Neuron"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0210","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1038\/nchembio.2461","article-title":"A toxic mutant huntingtin species is resistant to selective autophagy","volume":"13","author":"Fu","year":"2017","journal-title":"Nat. Chem. Biol."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0215","doi-asserted-by":"crossref","first-page":"3159","DOI":"10.1007\/s00018-018-2854-4","article-title":"Mechanisms of protein toxicity in neurodegenerative diseases","volume":"75","author":"Chung","year":"2018","journal-title":"Cell. Mol. Life Sci."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0220","doi-asserted-by":"crossref","first-page":"1553","DOI":"10.1016\/j.cell.2016.08.042","article-title":"Neuroendocrine coordination of mitochondrial stress signaling and proteostasis","volume":"166","author":"Berendzen","year":"2016","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0225","doi-asserted-by":"crossref","first-page":"7597","DOI":"10.1523\/JNEUROSCI.0990-06.2006","article-title":"Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model","volume":"26","author":"Brignull","year":"2006","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0230","doi-asserted-by":"crossref","first-page":"610","DOI":"10.2174\/1567205013666151221145445","article-title":"Evidence for mitochondrial UPR gene activation in familial and sporadic Alzheimer's disease","volume":"13","author":"S. Beck","year":"2016","journal-title":"Curr. Alzheimer Res."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0235","doi-asserted-by":"crossref","first-page":"11085","DOI":"10.1523\/JNEUROSCI.1294-17.2017","article-title":"Dysregulation of the mitochondrial unfolded protein response induces non-apoptotic dopaminergic neurodegeneration in C. elegans models of Parkinson's disease","volume":"37","author":"Martinez","year":"2017","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbadis.2019.02.015_bb0240","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1016\/j.ygeno.2014.08.022","article-title":"Regulation of the human ABCB10 gene by E2F transcription factors","volume":"104","author":"Karwaciak","year":"2014","journal-title":"Genomics"},{"key":"10.1016\/j.bbadis.2019.02.015_bb0245","doi-asserted-by":"crossref","first-page":"2492","DOI":"10.1093\/emboj\/19.11.2492","article-title":"ABC-me: a novel mitochondrial transporter induced by GATA-1 during erythroid differentiation","volume":"19","author":"Shirihai","year":"2000","journal-title":"EMBO J."}],"container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443919300675?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443919300675?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T04:52:36Z","timestamp":1760417556000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0925443919300675"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,6]]},"references-count":49,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2019,6]]}},"alternative-id":["S0925443919300675"],"URL":"https:\/\/doi.org\/10.1016\/j.bbadis.2019.02.015","relation":{},"ISSN":["0925-4439"],"issn-type":[{"value":"0925-4439","type":"print"}],"subject":[],"published":{"date-parts":[[2019,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Mutant huntingtin inhibits the mitochondrial unfolded protein response by impairing ABCB10 mRNA stability","name":"articletitle","label":"Article Title"},{"value":"Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bbadis.2019.02.015","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 Elsevier B.V.","name":"copyright","label":"Copyright"}]}}