{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T19:28:43Z","timestamp":1783366123707,"version":"3.54.6"},"reference-count":97,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,9,21]],"date-time":"2021-09-21T00:00:00Z","timestamp":1632182400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002243","name":"National Ataxia Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002243","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006710","name":"Wayne State University","doi-asserted-by":"publisher","award":["R01NS08677"],"award-info":[{"award-number":["R01NS08677"]}],"id":[{"id":"10.13039\/100006710","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2021,12]]},"DOI":"10.1016\/j.nbd.2021.105516","type":"journal-article","created":{"date-parts":[[2021,9,24]],"date-time":"2021-09-24T04:43:22Z","timestamp":1632458602000},"page":"105516","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":14,"special_numbering":"C","title":["Targeting the VCP-binding motif of ataxin-3 improves phenotypes in Drosophila models of Spinocerebellar Ataxia Type 3"],"prefix":"10.1016","volume":"160","author":[{"given":"Sean L.","family":"Johnson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kozeta","family":"Libohova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica R.","family":"Blount","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alyson L.","family":"Sujkowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew V.","family":"Prifti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei-Ling","family":"Tsou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sokol V.","family":"Todi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2021.105516_bb0005","doi-asserted-by":"crossref","first-page":"3155","DOI":"10.1111\/j.1432-1033.2004.04245.x","article-title":"Structural and functional analysis of ataxin-2 and ataxin-3","volume":"271","author":"Albrecht","year":"2004","journal-title":"Eur. J. Biochem."},{"key":"10.1016\/j.nbd.2021.105516_bb0010","doi-asserted-by":"crossref","first-page":"104697","DOI":"10.1016\/j.nbd.2019.104697","article-title":"Druggable genome screen identifies new regulators of the abundance and toxicity of ATXN3, the spinocerebellar Ataxia type 3 disease protein","volume":"137","author":"Ashraf","year":"2020","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2021.105516_bb0015","doi-asserted-by":"crossref","first-page":"32026","DOI":"10.1074\/jbc.M506084200","article-title":"Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3","volume":"280","author":"Berke","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0020","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0036542","article-title":"Ubiquitin-specific protease 25 functions in endoplasmic reticulum-associated degradation","volume":"7","author":"Blount","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2021.105516_bb0025","doi-asserted-by":"crossref","first-page":"4638","DOI":"10.1038\/ncomms5638","article-title":"Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23","volume":"5","author":"Blount","year":"2014","journal-title":"Nat. Commun."},{"key":"10.1016\/j.nbd.2021.105516_bb0030","doi-asserted-by":"crossref","first-page":"8513","DOI":"10.1038\/s41598-018-26364-x","article-title":"Expression and regulation of Deubiquitinase-resistant, unanchored ubiquitin chains in Drosophila","volume":"8","author":"Blount","year":"2018","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2021.105516_bb0035","doi-asserted-by":"crossref","DOI":"10.3390\/cells9061519","article-title":"Isoleucine 44 hydrophobic patch controls toxicity of unanchored, linear ubiquitin chains through NF-\u03baB signaling","volume":"9","author":"Blount","year":"2020","journal-title":"Cells."},{"key":"10.1016\/j.nbd.2021.105516_bb0040","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1038\/sj.emboj.7601043","article-title":"An arginine\/lysine-rich motif is crucial for VCP\/p97-mediated modulation of ataxin-3 fibrillogenesis","volume":"25","author":"Boeddrich","year":"2006","journal-title":"EMBO J."},{"key":"10.1016\/j.nbd.2021.105516_bb0045","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1016\/j.molcel.2010.10.001","article-title":"Protein quality control in the cytosol and the endoplasmic reticulum: brothers in arms","volume":"40","author":"Buchberger","year":"2010","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2021.105516_bb0050","doi-asserted-by":"crossref","first-page":"1100","DOI":"10.1002\/jnr.23395","article-title":"Using membrane-targeted green fluorescent protein to monitor neurotoxic protein-dependent degeneration of Drosophila eyes","volume":"92","author":"Burr","year":"2014","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.nbd.2021.105516_bb0055","first-page":"809","article-title":"Marked phenotypic heterogeneity associated with expansion of a CAG repeat sequence at the spinocerebellar ataxia 3\/Machado-Joseph disease locus","volume":"57","author":"Cancel","year":"1995","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0060","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1016\/j.ab.2007.09.015","article-title":"Detection and quantification of tau aggregation using a membrane filter assay","volume":"373","author":"Chang","year":"2008","journal-title":"Anal. Biochem."},{"key":"10.1016\/j.nbd.2021.105516_bb0065","article-title":"Identifying therapeutic targets for spinocerebellar Ataxia type 3\/Machado-Joseph disease through integration of pathological biomarkers and therapeutic strategies","volume":"21","author":"Chen","year":"2020","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.nbd.2021.105516_bb0070","doi-asserted-by":"crossref","first-page":"2891","DOI":"10.1093\/brain\/aww228","article-title":"Unbiased screen identifies aripiprazole as a modulator of abundance of the polyglutamine disease protein, ataxin-3","volume":"139","author":"Costa","year":"2016","journal-title":"Brain."},{"key":"10.1016\/j.nbd.2021.105516_bb0075","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/j.pneurobio.2011.11.006","article-title":"Toward understanding Machado-Joseph disease","volume":"97","author":"Costa","year":"2012","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.nbd.2021.105516_bb0080","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1212\/WNL.28.7.703","article-title":"Autosomal dominant system degeneration in Portuguese families of the Azores Islands. A new genetic disorder involving cerebellar, pyramidal, extrapyramidal and spinal cord motor functions","volume":"28","author":"Coutinho","year":"1978","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2021.105516_bb0085","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1007\/s13311-019-00798-1","article-title":"From pathogenesis to novel therapeutics for spinocerebellar Ataxia type 3: evading potholes on the way to translation","volume":"16","author":"Da Silva","year":"2019","journal-title":"Neurotherapeutics."},{"key":"10.1016\/j.nbd.2021.105516_bb0090","doi-asserted-by":"crossref","first-page":"3562","DOI":"10.1074\/jbc.273.6.3562","article-title":"Involvement of valosin-containing protein, an ATPase co-purified with IkappaBalpha and 26 S proteasome, in ubiquitin-proteasome-mediated degradation of IkappaBalpha","volume":"273","author":"Dai","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0095","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1007\/978-3-030-38266-7_10","article-title":"Machado-Joseph disease: a stress combating deubiquitylating enzyme changing sides","volume":"1233","author":"Dantuma","year":"2020","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.nbd.2021.105516_bb0100","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/j.molcel.2006.03.036","article-title":"Central pore residues mediate the p97\/VCP activity required for ERAD","volume":"22","author":"DeLaBarre","year":"2006","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2021.105516_bb0105","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.cell.2013.05.059","article-title":"Second cistron in CACNA1A gene encodes a transcription factor mediating cerebellar development and SCA6","volume":"154","author":"Du","year":"2013","journal-title":"Cell."},{"key":"10.1016\/j.nbd.2021.105516_bb0110","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1002\/ana.410390411","article-title":"Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features","volume":"39","author":"D\u00fcrr","year":"1996","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2021.105516_bb0115","doi-asserted-by":"crossref","first-page":"16888","DOI":"10.1074\/jbc.M601470200","article-title":"The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step","volume":"281","author":"Ellisdon","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0120","doi-asserted-by":"crossref","first-page":"2325","DOI":"10.1038\/nprot.2007.328","article-title":"Creating transgenic Drosophila by microinjecting the site-specific phiC31 integrase mRNA and a transgene-containing donor plasmid","volume":"2","author":"Fish","year":"2007","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.nbd.2021.105516_bb0125","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1093\/bioinformatics\/btv696","article-title":"SVD-phy: improved prediction of protein functional associations through singular value decomposition of phylogenetic profiles","volume":"32","author":"Franceschini","year":"2016","journal-title":"Bioinformatics."},{"key":"10.1016\/j.nbd.2021.105516_bb0130","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1002\/mds.10269","article-title":"Presumed rapid eye movement behavior disorder in Machado-Joseph disease (spinocerebellar ataxia type 3)","volume":"17","author":"Friedman","year":"2002","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105516_bb0135","doi-asserted-by":"crossref","first-page":"1520","DOI":"10.1002\/mds.10590","article-title":"REM behavior disorder and excessive daytime somnolence in Machado-Joseph disease (SCA-3)","volume":"18","author":"Friedman","year":"2003","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105516_bb0140","doi-asserted-by":"crossref","first-page":"1816","DOI":"10.1038\/ncomms2828","article-title":"A functional deficiency of TERA\/VCP\/p97 contributes to impaired DNA repair in multiple polyglutamine diseases","volume":"4","author":"Fujita","year":"2013","journal-title":"Nat. Commun."},{"key":"10.1016\/j.nbd.2021.105516_bb0145","first-page":"1775","article-title":"Construction of transgenic Drosophila by using the site-specific integrase from phage phiC31","volume":"166","author":"Groth","year":"2004","journal-title":"Genetics."},{"key":"10.1016\/j.nbd.2021.105516_bb0150","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0013695","article-title":"Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties","volume":"5","author":"Harris","year":"2010","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2021.105516_bb0155","doi-asserted-by":"crossref","first-page":"104535","DOI":"10.1016\/j.nbd.2019.104535","article-title":"Differential toxicity of ataxin-3 isoforms in Drosophila models of spinocerebellar Ataxia type 3","volume":"132","author":"Johnson","year":"2019","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2021.105516_bb0160","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.60742","article-title":"Ubiquitin-interacting motifs of ataxin-3 regulate its polyglutamine toxicity through Hsc70-4-dependent aggregation","volume":"9","author":"Johnson","year":"2020","journal-title":"Elife."},{"key":"10.1016\/j.nbd.2021.105516_bb0165","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ng1194-221","article-title":"CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1","volume":"8","author":"Kawaguchi","year":"1994","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0170","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":"Kim","year":"2013","journal-title":"Neuron."},{"key":"10.1016\/j.nbd.2021.105516_bb0175","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1038\/s41572-019-0074-3","article-title":"Spinocerebellar ataxia","volume":"5","author":"Klockgether","year":"2019","journal-title":"Nat Rev Dis Primers."},{"key":"10.1016\/j.nbd.2021.105516_bb0180","doi-asserted-by":"crossref","first-page":"47358","DOI":"10.1074\/jbc.M207783200","article-title":"Functional ATPase activity of p97\/valosin-containing protein (VCP) is required for the quality control of endoplasmic reticulum in neuronally differentiated mammalian PC12 cells","volume":"277","author":"Kobayashi","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0185","doi-asserted-by":"crossref","first-page":"D899","DOI":"10.1093\/nar\/gkaa1026","article-title":"FlyBase: updates to the Drosophila melanogaster knowledge base","volume":"49","author":"Larkin","year":"2021","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2021.105516_bb0190","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.pneurobio.2015.06.004","article-title":"Toward therapeutic targets for SCA3: insight into the role of Machado-Joseph disease protein ataxin-3 in misfolded proteins clearance","volume":"132","author":"Li","year":"2015","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.nbd.2021.105516_bb0195","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1146\/annurev-pathmechdis-012418-012857","article-title":"Polyglutamine repeats in neurodegenerative diseases","volume":"14","author":"Lieberman","year":"2019","journal-title":"Annu. Rev. Pathol."},{"key":"10.1016\/j.nbd.2021.105516_bb0200","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1159\/000066169","article-title":"Peripheral neuropathy of Machado-Joseph disease in Taiwan: a morphometric and genetic study","volume":"48","author":"Lin","year":"2002","journal-title":"Eur. Neurol."},{"key":"10.1016\/j.nbd.2021.105516_bb0205","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1016\/j.jmb.2004.09.065","article-title":"Characterization of the structure and the amyloidogenic properties of the Josephin domain of the polyglutamine-containing protein ataxin-3","volume":"344","author":"Masino","year":"2004","journal-title":"J. Mol. Biol."},{"key":"10.1016\/j.nbd.2021.105516_bb0210","doi-asserted-by":"crossref","first-page":"2033","DOI":"10.1016\/j.bpj.2011.02.056","article-title":"The Josephin domain determines the morphological and mechanical properties of ataxin-3 fibrils","volume":"100","author":"Masino","year":"2011","journal-title":"Biophys. J."},{"key":"10.1016\/j.nbd.2021.105516_bb0215","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.pneurobio.2011.06.007","article-title":"Polyglutamine diseases: the special case of ataxin-3 and Machado-Joseph disease","volume":"95","author":"Matos","year":"2011","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.nbd.2021.105516_bb0220","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1111\/jnc.14541","article-title":"Machado-Joseph disease\/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy","volume":"148","author":"Matos","year":"2019","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2021.105516_bb0225","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/0022-510X(96)00016-0","article-title":"The relationship between trinucleotide repeat length and phenotypic variation in Machado-Joseph disease","volume":"139","author":"Matsumura","year":"1996","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.nbd.2021.105516_bb0230","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1038\/ncb2407","article-title":"Emerging functions of the VCP\/p97 AAA-ATPase in the ubiquitin system","volume":"14","author":"Meyer","year":"2012","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.nbd.2021.105516_bb0235","doi-asserted-by":"crossref","first-page":"101504","DOI":"10.1016\/j.scr.2019.101504","article-title":"Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line","volume":"39","author":"Moore","year":"2019","journal-title":"Stem Cell Res."},{"key":"10.1016\/j.nbd.2021.105516_bb0240","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1111\/j.1365-2443.2009.01286.x","article-title":"p97\/valosin-containing protein (VCP) is highly modulated by phosphorylation and acetylation","volume":"14","author":"Mori-Konya","year":"2009","journal-title":"Genes Cells"},{"key":"10.1016\/j.nbd.2021.105516_bb0245","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1111\/j.1742-4658.2008.06858.x","article-title":"Evolutionary divergence of valosin-containing protein\/cell division cycle protein 48 binding interactions among endoplasmic reticulum-associated degradation proteins","volume":"276","author":"Morreale","year":"2009","journal-title":"FEBS J."},{"key":"10.1016\/j.nbd.2021.105516_bb0250","doi-asserted-by":"crossref","first-page":"78","DOI":"10.3389\/fnmol.2017.00078","article-title":"The ubiquitination, disaggregation and proteasomal degradation machineries in Polyglutamine disease","volume":"10","author":"Nath","year":"2017","journal-title":"Front. Mol. Neurosci."},{"key":"10.1016\/j.nbd.2021.105516_bb0255","doi-asserted-by":"crossref","first-page":"1203","DOI":"10.1002\/bip.21210","article-title":"Josephin domain of ataxin-3 contains two distinct ubiquitin-binding sites","volume":"91","author":"Nicastro","year":"2009","journal-title":"Biopolymers."},{"key":"10.1016\/j.nbd.2021.105516_bb0260","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0012430","article-title":"Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3","volume":"5","author":"Nicastro","year":"2010","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2021.105516_bb0265","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1007\/s12041-018-0957-1","article-title":"Huntington\u2019s disease: the coming of age","volume":"97","author":"Pandey","year":"2018","journal-title":"J. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0270","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/B978-0-444-51892-7.00027-9","article-title":"Machado-Joseph disease\/spinocerebellar ataxia type 3","volume":"103","author":"Paulson","year":"2012","journal-title":"Handb. Clin. Neurol."},{"key":"10.1016\/j.nbd.2021.105516_bb0275","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1002\/ana.410410408","article-title":"Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain","volume":"41","author":"Paulson","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2021.105516_bb0280","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1038\/nrn.2017.92","article-title":"Polyglutamine spinocerebellar ataxias - from genes to potential treatments","volume":"18","author":"Paulson","year":"2017","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.nbd.2021.105516_bb0285","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1007\/978-3-319-71779-1_6","article-title":"Spinocerebellar Ataxia type 1: molecular mechanisms of neurodegeneration and preclinical studies","volume":"1049","author":"P\u00e9rez Ortiz","year":"2018","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.nbd.2021.105516_bb0290","first-page":"603","article-title":"Spinocerebellar ataxia type 1 and Machado-Joseph disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia","volume":"57","author":"Ranum","year":"1995","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0295","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1016\/j.nbd.2018.04.013","article-title":"Toxicity and aggregation of the polyglutamine disease protein, ataxin-3 is regulated by its binding to VCP\/p97 in Drosophila melanogaster","volume":"116","author":"Ristic","year":"2018","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2021.105516_bb0300","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1002\/mds.870070302","article-title":"Machado-Joseph disease: an autosomal dominant motor system degeneration","volume":"7","author":"Rosenberg","year":"1992","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105516_bb0305","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/S0006-8993(02)03278-X","article-title":"Degeneration of the external cuneate nucleus in spinocerebellar ataxia type 3 (Machado-Joseph disease)","volume":"953","author":"R\u00fcb","year":"2002","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2021.105516_bb0310","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.1093\/brain\/awf208","article-title":"Spinocerebellar ataxia type 3 (Machado-Joseph disease): severe destruction of the lateral reticular nucleus","volume":"125","author":"R\u00fcb","year":"2002","journal-title":"Brain."},{"key":"10.1016\/j.nbd.2021.105516_bb0315","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/S0891-0618(02)00099-6","article-title":"The nucleus raphe interpositus in spinocerebellar ataxia type 3 (Machado-Joseph disease)","volume":"25","author":"R\u00fcb","year":"2003","journal-title":"J. Chem. Neuroanat."},{"key":"10.1016\/j.nbd.2021.105516_bb0320","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1111\/j.1365-2990.2004.00554.x","article-title":"Degeneration of the central vestibular system in spinocerebellar ataxia type 3 (SCA3) patients and its possible clinical significance","volume":"30","author":"R\u00fcb","year":"2004","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.nbd.2021.105516_bb0325","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1212\/01.WNL.0000140498.24112.8C","article-title":"Damage to the reticulotegmental nucleus of the pons in spinocerebellar ataxia type 1, 2, and 3","volume":"63","author":"R\u00fcb","year":"2004","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2021.105516_bb0330","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/0022-510X(95)00175-2","article-title":"CAG repeat expansion of Machado-Joseph disease in the Japanese: analysis of the repeat instability for parental transmission, and correlation with disease phenotype","volume":"133","author":"Sasaki","year":"1995","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.nbd.2021.105516_bb0335","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/j.molcel.2011.05.036","article-title":"Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP","volume":"43","author":"Scaglione","year":"2011","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2021.105516_bb0340","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1016\/j.bbrc.2007.08.062","article-title":"Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination","volume":"362","author":"Schmitt","year":"2007","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.nbd.2021.105516_bb0345","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1136\/jnnp.61.5.466","article-title":"Relations between genotype and phenotype in German patients with the Machado-Joseph disease mutation","volume":"61","author":"Sch\u00f6ls","year":"1996","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.nbd.2021.105516_bb0350","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1007\/978-3-7091-0579-5_16","article-title":"Therapeutic strategies in Friedreich\u2019s ataxia","author":"Sch\u00f6ls","year":"2004","journal-title":"J. Neural Transm. Suppl."},{"key":"10.1016\/j.nbd.2021.105516_bb0355","first-page":"139","article-title":"Epidemiology and clinical aspects of Machado-Joseph disease","volume":"61","author":"Sequeiros","year":"1993","journal-title":"Adv. Neurol."},{"key":"10.1016\/j.nbd.2021.105516_bb0360","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1002\/ana.410410407","article-title":"Machado-Joseph disease: clinical, molecular, and metabolic characterization in Chinese kindreds","volume":"41","author":"Soong","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2021.105516_bb0365","doi-asserted-by":"crossref","first-page":"535","DOI":"10.18632\/aging.100789","article-title":"Endurance exercise and selective breeding for longevity extend Drosophila healthspan by overlapping mechanisms","volume":"7","author":"Sujkowski","year":"2015","journal-title":"Aging (Albany NY)"},{"key":"10.1016\/j.nbd.2021.105516_bb0370","doi-asserted-by":"crossref","first-page":"1419","DOI":"10.1093\/hmg\/ddx039","article-title":"Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of spinocerebellar Ataxia type 3","volume":"26","author":"Sutton","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0375","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1007\/s12017-010-8137-3","article-title":"Mouse ataxin-3 functional knock-out model","volume":"13","author":"Switonski","year":"2011","journal-title":"NeuroMolecular Med."},{"key":"10.1016\/j.nbd.2021.105516_bb0380","doi-asserted-by":"crossref","first-page":"D362","DOI":"10.1093\/nar\/gkw937","article-title":"The STRING database in 2017: quality-controlled protein-protein association networks, made broadly accessible","volume":"45","author":"Szklarczyk","year":"2017","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2021.105516_bb0385","doi-asserted-by":"crossref","first-page":"D607","DOI":"10.1093\/nar\/gky1131","article-title":"STRING v11: protein-protein association networks with increased coverage, supporting functional discovery in genome-wide experimental datasets","volume":"47","author":"Szklarczyk","year":"2019","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2021.105516_bb0390","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1038\/ng0793-300","article-title":"The gene for Machado-Joseph disease maps to human chromosome 14q","volume":"4","author":"Takiyama","year":"1993","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0395","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1212\/WNL.44.7.1302","article-title":"A clinical and pathologic study of a large Japanese family with Machado-Joseph disease tightly linked to the DNA markers on chromosome 14q","volume":"44","author":"Takiyama","year":"1994","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2021.105516_bb0400","series-title":"Molecular Neurology","first-page":"257","article-title":"Polyglutamine disorders including huntington\u2019s disease","author":"Todi","year":"2007"},{"key":"10.1016\/j.nbd.2021.105516_bb0405","doi-asserted-by":"crossref","first-page":"29348","DOI":"10.1074\/jbc.M704126200","article-title":"Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity","volume":"282","author":"Todi","year":"2007","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0410","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1038\/emboj.2008.289","article-title":"Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3","volume":"28","author":"Todi","year":"2009","journal-title":"EMBO J."},{"key":"10.1016\/j.nbd.2021.105516_bb0415","doi-asserted-by":"crossref","first-page":"34460","DOI":"10.1074\/jbc.M113.513903","article-title":"Ubiquitination regulates the neuroprotective function of the deubiquitinase ataxin-3 in vivo","volume":"288","author":"Tsou","year":"2013","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0420","doi-asserted-by":"crossref","first-page":"4385","DOI":"10.1093\/hmg\/ddv174","article-title":"DnaJ-1 and karyopherin \u03b13 suppress degeneration in a new Drosophila model of spinocerebellar Ataxia type 6","volume":"24","author":"Tsou","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0425","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.nbd.2015.05.010","article-title":"The deubiquitinase ataxin-3 requires Rad23 and DnaJ-1 for its neuroprotective role in Drosophila melanogaster","volume":"82","author":"Tsou","year":"2015","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2021.105516_bb0430","doi-asserted-by":"crossref","first-page":"1770","DOI":"10.1242\/bio.021667","article-title":"Polyglutamine length-dependent toxicity from \u03b11ACT in Drosophila models of spinocerebellar ataxia type 6","volume":"5","author":"Tsou","year":"2016","journal-title":"Biol Open."},{"key":"10.1016\/j.nbd.2021.105516_bb0435","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/S0006-291X(02)02840-1","article-title":"Hexamerization of p97-VCP is promoted by ATP binding to the D1 domain and required for ATPase and biological activities","volume":"300","author":"Wang","year":"2003","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.nbd.2021.105516_bb0440","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1083\/jcb.200605100","article-title":"Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3","volume":"174","author":"Wang","year":"2006","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2021.105516_bb0445","doi-asserted-by":"crossref","first-page":"7445","DOI":"10.1074\/jbc.M708347200","article-title":"Inhibition of p97-dependent protein degradation by Eeyarestatin I","volume":"283","author":"Wang","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0450","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1111\/j.1399-0004.1998.tb02575.x","article-title":"Frequency analysis of autosomal dominant cerebellar ataxias in Japanese patients and clinical characterization of spinocerebellar ataxia type 6","volume":"53","author":"Watanabe","year":"1998","journal-title":"Clin. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0455","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1074\/jbc.RA118.005801","article-title":"Physiological and pathophysiological characteristics of ataxin-3 isoforms","volume":"294","author":"Weish\u00e4upl","year":"2019","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0460","doi-asserted-by":"crossref","first-page":"26436","DOI":"10.1074\/jbc.M803692200","article-title":"The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains","volume":"283","author":"Winborn","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105516_bb0465","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1097\/00002093-200207000-00010","article-title":"Rapid detection of protein aggregates in the brains of Alzheimer patients and transgenic mouse models of amyloidosis","volume":"16","author":"Xu","year":"2002","journal-title":"Alzheimer Dis. Assoc. Disord."},{"key":"10.1016\/j.nbd.2021.105516_bb0470","doi-asserted-by":"crossref","first-page":"201","DOI":"10.3233\/JHD-130058","article-title":"The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease","volume":"2","author":"Zeng","year":"2013","journal-title":"J Huntingtons Dis."},{"key":"10.1016\/j.nbd.2021.105516_bb0475","doi-asserted-by":"crossref","first-page":"2409","DOI":"10.1093\/hmg\/ddl164","article-title":"Ataxin-3 binds VCP\/p97 and regulates retrotranslocation of ERAD substrates","volume":"15","author":"Zhong","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2021.105516_bb0480","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1212\/WNL.48.2.482","article-title":"Machado-Joseph disease in four Chinese pedigrees: molecular analysis of 15 patients including two juvenile cases and clinical correlations","volume":"48","author":"Zhou","year":"1997","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2021.105516_bb0485","doi-asserted-by":"crossref","first-page":"7425","DOI":"10.1074\/jbc.R800041200","article-title":"Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1","volume":"284","author":"Zoghbi","year":"2009","journal-title":"J. Biol. Chem."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996121002655?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996121002655?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T05:27:22Z","timestamp":1760851642000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996121002655"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":97,"alternative-id":["S0969996121002655"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2021.105516","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2021,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Targeting the VCP-binding motif of ataxin-3 improves phenotypes in Drosophila models of Spinocerebellar Ataxia Type 3","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2021.105516","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"105516"}}