{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T06:13:56Z","timestamp":1772691236354,"version":"3.50.1"},"publisher-location":"Cham","reference-count":159,"publisher":"Springer International Publishing","isbn-type":[{"value":"9783030382650","type":"print"},{"value":"9783030382667","type":"electronic"}],"license":[{"start":{"date-parts":[[2020,1,1]],"date-time":"2020-01-01T00:00:00Z","timestamp":1577836800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020]]},"DOI":"10.1007\/978-3-030-38266-7_10","type":"book-chapter","created":{"date-parts":[[2020,4,9]],"date-time":"2020-04-09T09:03:01Z","timestamp":1586422981000},"page":"237-260","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Machado\u2013Joseph Disease: A Stress Combating Deubiquitylating Enzyme Changing Sides"],"prefix":"10.1007","author":[{"given":"Nico P.","family":"Dantuma","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura K.","family":"Herzog","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,4,10]]},"reference":[{"issue":"1","key":"10_CR1","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1212\/WNL.22.1.49","volume":"22","author":"KK Nakano","year":"1972","unstructured":"Nakano KK, Dawson DM, Spence A (1972) Machado disease. A hereditary ataxia in Portuguese emigrants to Massachusetts. Neurology 22(1):49\u201355","journal-title":"Neurology"},{"issue":"26","key":"10_CR2","doi-asserted-by":"publisher","first-page":"1505","DOI":"10.1056\/NEJM197706302962606","volume":"296","author":"FC Romanul","year":"1977","unstructured":"Romanul FC, Fowler HL, Radvany J, Feldman RG, Feingold M (1977) Azorean disease of the nervous system. N Engl J Med 296(26):1505\u20131508","journal-title":"N Engl J Med"},{"issue":"8","key":"10_CR3","doi-asserted-by":"publisher","first-page":"703","DOI":"10.1212\/WNL.26.8.703","volume":"26","author":"RN Rosenberg","year":"1976","unstructured":"Rosenberg RN, Nyhan WL, Bay C, Shore P (1976) Autosomal dominant striatonigral degeneration. A clinical, pathologic, and biochemical study of a new genetic disorder. Neurology 26(8):703\u2013714","journal-title":"Neurology"},{"issue":"2","key":"10_CR4","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1016\/0022-510X(72)90137-2","volume":"17","author":"BT Woods","year":"1972","unstructured":"Woods BT, Schaumburg HH (1972) Nigro-spino-dentatal degeneration with nuclear ophthalmoplegia. A unique and partially treatable clinico-pathological entity. J Neurolog Sci 17(2):149\u2013166","journal-title":"J Neurolog Sci"},{"issue":"8","key":"10_CR5","doi-asserted-by":"publisher","first-page":"641","DOI":"10.1038\/nrn1474","volume":"5","author":"F Taroni","year":"2004","unstructured":"Taroni F, DiDonato S (2004) Pathways to motor incoordination: the inherited ataxias. Nat Rev Neurosci 5(8):641\u2013655","journal-title":"Nat Rev Neurosci"},{"issue":"6","key":"10_CR6","doi-asserted-by":"publisher","first-page":"1001","DOI":"10.1093\/hmg\/4.6.1001","volume":"4","author":"L Schols","year":"1995","unstructured":"Schols L, Vieira-Saecker AM, Schols S, Przuntek H, Epplen JT, Riess O (1995) Trinucleotide expansion within the MJD1 gene presents clinically as spinocerebellar ataxia and occurs most frequently in German SCA patients. Hum Mol Genet 4(6):1001\u20131005","journal-title":"Hum Mol Genet"},{"key":"10_CR7","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1146\/annurev.neuro.29.051605.113042","volume":"30","author":"HT Orr","year":"2007","unstructured":"Orr HT, Zoghbi HY (2007) Trinucleotide repeat disorders. Annu Rev Neurosci 30:575\u2013621","journal-title":"Annu Rev Neurosci"},{"issue":"9","key":"10_CR8","doi-asserted-by":"publisher","first-page":"921","DOI":"10.1001\/archneur.1984.04050200027013","volume":"41","author":"HL Fowler","year":"1984","unstructured":"Fowler HL (1984) Machado-Joseph-Azorean disease. A ten-year study. Arch Neurol 41(9):921\u2013925","journal-title":"Arch Neurol"},{"issue":"6","key":"10_CR9","doi-asserted-by":"publisher","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","volume":"72","author":"Group THsDCR","year":"1993","unstructured":"Group THsDCR (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes. Cell 72(6):971\u2013983","journal-title":"Cell"},{"issue":"1","key":"10_CR10","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1038\/ng0194-9","volume":"6","author":"R Koide","year":"1994","unstructured":"Koide R, Ikeuchi T, Onodera O, Tanaka H, Igarashi S, Endo K et al (1994) Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Nat Genet 6(1):9\u201313","journal-title":"Nat Genet"},{"issue":"6330","key":"10_CR11","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1038\/352077a0","volume":"352","author":"AR Spada La","year":"1991","unstructured":"La Spada AR, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH (1991) Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature (London) 352(6330):77\u201379","journal-title":"Nature (London)"},{"issue":"1","key":"10_CR12","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1038\/ng0194-14","volume":"6","author":"S Nagafuchi","year":"1994","unstructured":"Nagafuchi S, Yanagisawa H, Sato K, Shirayama T, Ohsaki E, Bundo M et al (1994) Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p. Nat Genet 6(1):14\u201318","journal-title":"Nat Genet"},{"issue":"3","key":"10_CR13","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1038\/ng0793-221","volume":"4","author":"HT Orr","year":"1993","unstructured":"Orr HT, Chung MY, Banfi S, Kwiatkowski TJ Jr, Servadio A, Beaudet AL et al (1993) Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nat Genet 4(3):221\u2013226","journal-title":"Nat Genet"},{"issue":"3","key":"10_CR14","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1038\/ng1194-221","volume":"8","author":"Y Kawaguchi","year":"1994","unstructured":"Kawaguchi Y, Okamoto T, Taniwaki M, Aizawa M, Inoue M, Katayama S et al (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat Genet 8(3):221\u2013228","journal-title":"Nat Genet"},{"issue":"4","key":"10_CR15","doi-asserted-by":"publisher","first-page":"453","DOI":"10.1002\/ana.410410408","volume":"41","author":"HL Paulson","year":"1997","unstructured":"Paulson HL, Das SS, Crino PB, Perez MK, Patel SC, Gotsdiner D et al (1997) Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain. Ann Neurol 41(4):453\u2013462","journal-title":"Ann Neurol"},{"issue":"2","key":"10_CR16","doi-asserted-by":"publisher","first-page":"333","DOI":"10.1016\/S0896-6273(00)80943-5","volume":"19","author":"HL Paulson","year":"1997","unstructured":"Paulson HL, Perez MK, Trottier Y, Trojanowski JQ, Subramony SH, Das SS et al (1997) Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19(2):333\u2013344","journal-title":"Neuron"},{"issue":"7","key":"10_CR17","doi-asserted-by":"publisher","first-page":"1137","DOI":"10.1093\/hmg\/4.7.1137","volume":"4","author":"Y Takiyama","year":"1995","unstructured":"Takiyama Y, Igarashi S, Rogaeva EA, Endo K, Rogaev EI, Tanaka H et al (1995) Evidence for inter-generational instability in the CAG repeat in the MJD1 gene and for conserved haplotypes at flanking markers amongst Japanese and Caucasian subjects with Machado-Joseph disease. Hum Mol Genet 4(7):1137\u20131146","journal-title":"Hum Mol Genet"},{"issue":"1","key":"10_CR18","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1016\/S0896-6273(01)00177-5","volume":"29","author":"MY Sherman","year":"2001","unstructured":"Sherman MY, Goldberg AL (2001) Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases. Neuron 29(1):15\u201332","journal-title":"Neuron"},{"issue":"1","key":"10_CR19","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1016\/j.molcel.2018.02.024","volume":"70","author":"P Haahr","year":"2018","unstructured":"Haahr P, Borgermann N, Guo X, Typas D, Achuthankutty D, Hoffmann S et al (2018) ZUFSP deubiquitylates K63-linked polyubiquitin chains to promote genome stability. Mol Cell 70(1):165\u2013174. e6","journal-title":"Mol Cell"},{"issue":"8","key":"10_CR20","doi-asserted-by":"publisher","first-page":"550","DOI":"10.1038\/nrm2731","volume":"10","author":"D Komander","year":"2009","unstructured":"Komander D, Clague MJ, Urbe S (2009) Breaking the chains: structure and function of the deubiquitinases. Nat Rev Mol Cell Biol 10(8):550\u2013563","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"1","key":"10_CR21","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1016\/j.molcel.2018.02.023","volume":"70","author":"D Kwasna","year":"2018","unstructured":"Kwasna D, Abdul Rehman SA, Natarajan J, Matthews S, Madden R, De Cesare V et al (2018) Discovery and characterization of ZUFSP\/ZUP1, a distinct deubiquitinase class important for genome stability. Mol Cell 70(1):150\u2013164","journal-title":"Mol Cell"},{"issue":"Pt 2","key":"10_CR22","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1242\/jcs.090985","volume":"125","author":"MJ Clague","year":"2012","unstructured":"Clague MJ, Coulson JM, Urbe S (2012) Cellular functions of the DUBs. J Cell Sci 125(Pt 2):277\u2013286","journal-title":"J Cell Sci"},{"key":"10_CR23","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1146\/annurev.biochem.67.1.425","volume":"67","author":"A Hershko","year":"1998","unstructured":"Hershko A, Ciechanover A (1998) The ubiquitin system. Annu Rev Biochem 67:425\u2013479","journal-title":"Annu Rev Biochem"},{"key":"10_CR24","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1146\/annurev.biochem.70.1.503","volume":"70","author":"CM Pickart","year":"2001","unstructured":"Pickart CM (2001) Mechanisms underlying ubiquitination. Annu Rev Biochem 70:503\u2013533","journal-title":"Annu Rev Biochem"},{"key":"10_CR25","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1146\/annurev-biochem-060310-170328","volume":"81","author":"D Komander","year":"2012","unstructured":"Komander D, Rape M (2012) The ubiquitin code. Annu Rev Biochem 81:203\u2013229","journal-title":"Annu Rev Biochem"},{"issue":"5","key":"10_CR26","doi-asserted-by":"publisher","first-page":"779","DOI":"10.1016\/j.molcel.2017.02.011","volume":"65","author":"G Dittmar","year":"2017","unstructured":"Dittmar G, Selbach M (2017) Deciphering the ubiquitin code. Mol Cell 65(5):779\u2013780","journal-title":"Mol Cell"},{"issue":"8","key":"10_CR27","doi-asserted-by":"publisher","first-page":"5501","DOI":"10.1128\/MCB.14.8.5501","volume":"14","author":"D Finley","year":"1994","unstructured":"Finley D, Sadis S, Monia BP, Boucher P, Ecker DJ, Crooke ST et al (1994) Inhibition of proteolysis and cell cycle progression in a multiubiquitination-deficient yeast mutant. Mol Cell Biol 14(8):5501\u20135509","journal-title":"Mol Cell Biol"},{"issue":"4","key":"10_CR28","doi-asserted-by":"publisher","first-page":"552","DOI":"10.1038\/emboj.2012.354","volume":"32","author":"JA Nathan","year":"2013","unstructured":"Nathan JA, Kim HT, Ting L, Gygi SP, Goldberg AL (2013) Why do cellular proteins linked to K63-polyubiquitin chains not associate with proteasomes? EMBO J 32(4):552\u2013565","journal-title":"EMBO J"},{"issue":"17","key":"10_CR29","doi-asserted-by":"publisher","first-page":"7704","DOI":"10.1073\/pnas.0908764107","volume":"107","author":"S Zhao","year":"2010","unstructured":"Zhao S, Ulrich HD (2010) Distinct consequences of posttranslational modification by linear versus K63-linked polyubiquitin chains. Proc Natl Acad Sci U S A 107(17):7704\u20137709","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"8","key":"10_CR30","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1038\/nrm1700","volume":"6","author":"RL Welchman","year":"2005","unstructured":"Welchman RL, Gordon C, Mayer RJ (2005) Ubiquitin and ubiquitin-like proteins as multifunctional signals. Nat Rev Mol Cell Biol 6(8):599\u2013609","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"23","key":"10_CR31","doi-asserted-by":"publisher","first-page":"3195","DOI":"10.1093\/hmg\/ddg344","volume":"12","author":"B Burnett","year":"2003","unstructured":"Burnett B, Li F, Pittman RN (2003) The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. Hum Mol Genet 12(23):3195\u20133205","journal-title":"Hum Mol Genet"},{"issue":"10","key":"10_CR32","doi-asserted-by":"publisher","first-page":"5461","DOI":"10.1074\/jbc.273.10.5461","volume":"273","author":"P Young","year":"1998","unstructured":"Young P, Deveraux Q, Beal RE, Pickart CM, Rechsteiner M (1998) Characterization of two polyubiquitin binding sites in the 26 S protease subunit 5a. J Biol Chem 273(10):5461\u20135467","journal-title":"J Biol Chem"},{"issue":"4","key":"10_CR33","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1016\/S0168-0102(97)00056-4","volume":"28","author":"J Goto","year":"1997","unstructured":"Goto J, Watanabe M, Ichikawa Y, Yee SB, Ihara N, Endo K et al (1997) Machado-Joseph disease gene products carrying different carboxyl termini. Neurosci Res 28(4):373\u2013377","journal-title":"Neurosci Res"},{"issue":"4","key":"10_CR34","doi-asserted-by":"publisher","first-page":"669","DOI":"10.1111\/j.1750-3639.1998.tb00193.x","volume":"8","author":"T Schmidt","year":"1998","unstructured":"Schmidt T, Landwehrmeyer GB, Schmitt I, Trottier Y, Auburger G, Laccone F et al (1998) An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients. Brain Pathol 8(4):669\u2013679","journal-title":"Brain Pathol"},{"issue":"10","key":"10_CR35","doi-asserted-by":"publisher","first-page":"e13695","DOI":"10.1371\/journal.pone.0013695","volume":"5","author":"GM Harris","year":"2010","unstructured":"Harris GM, Dodelzon K, Gong L, Gonzalez-Alegre P, Paulson HL (2010) Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. PLoS One 5(10):e13695","journal-title":"PLoS One"},{"issue":"15","key":"10_CR36","doi-asserted-by":"publisher","first-page":"8892","DOI":"10.1073\/pnas.1530212100","volume":"100","author":"KM Donaldson","year":"2003","unstructured":"Donaldson KM, Li W, Ching KA, Batalov S, Tsai CC, Joazeiro CA (2003) Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates. Proc Natl Acad Sci U S A 100(15):8892\u20138897","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"7","key":"10_CR37","doi-asserted-by":"publisher","first-page":"1883","DOI":"10.1083\/jcb.143.7.1883","volume":"143","author":"JA Johnston","year":"1998","unstructured":"Johnston JA, Ward CL, Kopito RR (1998) Aggresomes: a cellular response to misfolded proteins. J Cell Biol 143(7):1883\u20131898","journal-title":"J Cell Biol"},{"issue":"7010","key":"10_CR38","doi-asserted-by":"publisher","first-page":"805","DOI":"10.1038\/nature02998","volume":"431","author":"M Arrasate","year":"2004","unstructured":"Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S (2004) Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature (London) 431(7010):805\u2013810","journal-title":"Nature (London)"},{"issue":"1","key":"10_CR39","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1093\/hmg\/ddq452","volume":"20","author":"TM Durcan","year":"2011","unstructured":"Durcan TM, Kontogiannea M, Thorarinsdottir T, Fallon L, Williams AJ, Djarmati A et al (2011) The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability. Hum Mol Genet 20(1):141\u2013154","journal-title":"Hum Mol Genet"},{"issue":"6676","key":"10_CR40","doi-asserted-by":"publisher","first-page":"605","DOI":"10.1038\/33416","volume":"392","author":"T Kitada","year":"1998","unstructured":"Kitada T, Asakawa S, Hattori N, Matsumine H, Yamamura Y, Minoshima S et al (1998) Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. Nature (London) 392(6676):605\u2013608","journal-title":"Nature (London)"},{"issue":"2","key":"10_CR41","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1038\/ncb2407","volume":"14","author":"H Meyer","year":"2012","unstructured":"Meyer H, Bug M, Bremer S (2012) Emerging functions of the VCP\/p97 AAA-ATPase in the ubiquitin system. Nat Cell Biol 14(2):117\u2013123","journal-title":"Nat Cell Biol"},{"issue":"4","key":"10_CR42","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1038\/ng1332","volume":"36","author":"GD Watts","year":"2004","unstructured":"Watts GD, Wymer J, Kovach MJ, Mehta SG, Mumm S, Darvish D et al (2004) Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nat Genet 36(4):377\u2013381","journal-title":"Nat Genet"},{"issue":"5","key":"10_CR43","doi-asserted-by":"publisher","first-page":"857","DOI":"10.1016\/j.neuron.2010.11.036","volume":"68","author":"JO Johnson","year":"2010","unstructured":"Johnson JO, Mandrioli J, Benatar M, Abramzon Y, Van Deerlin VM, Trojanowski JQ et al (2010) Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron 68(5):857\u2013864","journal-title":"Neuron"},{"issue":"21","key":"10_CR44","doi-asserted-by":"publisher","first-page":"2845","DOI":"10.1093\/hmg\/ddg297","volume":"12","author":"H Scheel","year":"2003","unstructured":"Scheel H, Tomiuk S, Hofmann K (2003) Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics. Hum Mol Genet 12(21):2845\u20132852","journal-title":"Hum Mol Genet"},{"issue":"39","key":"10_CR45","doi-asserted-by":"publisher","first-page":"26436","DOI":"10.1074\/jbc.M803692200","volume":"283","author":"BJ Winborn","year":"2008","unstructured":"Winborn BJ, Travis SM, Todi SV, Scaglione KM, Xu P, Williams AJ et al (2008) The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. J Biol Chem 283(39):26436\u201326443","journal-title":"J Biol Chem"},{"issue":"4","key":"10_CR46","doi-asserted-by":"publisher","first-page":"372","DOI":"10.1038\/emboj.2008.289","volume":"28","author":"SV Todi","year":"2009","unstructured":"Todi SV, Winborn BJ, Scaglione KM, Blount JR, Travis SM, Paulson HL (2009) Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3. EMBO J 28(4):372\u2013382","journal-title":"EMBO J"},{"issue":"6","key":"10_CR47","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1016\/j.molcel.2009.02.011","volume":"33","author":"JJ Sims","year":"2009","unstructured":"Sims JJ, Cohen RE (2009) Linkage-specific avidity defines the lysine 63-linked polyubiquitin-binding preference of rap80. Mol Cell 33(6):775\u2013783","journal-title":"Mol Cell"},{"issue":"12","key":"10_CR48","doi-asserted-by":"publisher","first-page":"1203","DOI":"10.1002\/bip.21210","volume":"91","author":"G Nicastro","year":"2009","unstructured":"Nicastro G, Masino L, Esposito V, Menon RP, De Simone A, Fraternali F et al (2009) Josephin domain of ataxin-3 contains two distinct ubiquitin-binding sites. Biopolymers 91(12):1203\u20131214","journal-title":"Biopolymers"},{"issue":"8","key":"10_CR49","doi-asserted-by":"publisher","first-page":"e12430","DOI":"10.1371\/journal.pone.0012430","volume":"5","author":"G Nicastro","year":"2010","unstructured":"Nicastro G, Todi SV, Karaca E, Bonvin AM, Paulson HL, Pastore A (2010) Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3. PLoS One 5(8):e12430","journal-title":"PLoS One"},{"issue":"4","key":"10_CR50","doi-asserted-by":"publisher","first-page":"449","DOI":"10.1016\/j.dnarep.2009.01.005","volume":"8","author":"NP Dantuma","year":"2009","unstructured":"Dantuma NP, Heinen C, Hoogstraten D (2009) The ubiquitin receptor Rad23: at the crossroads of nucleotide excision repair and proteasomal degradation. DNA Repair 8(4):449\u2013460","journal-title":"DNA Repair"},{"key":"10_CR51","doi-asserted-by":"publisher","first-page":"4638","DOI":"10.1038\/ncomms5638","volume":"5","author":"JR Blount","year":"2014","unstructured":"Blount JR, Tsou WL, Ristic G, Burr AA, Ouyang M, Galante H et al (2014) Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23. Nat Commun 5:4638","journal-title":"Nat Commun"},{"issue":"13","key":"10_CR52","doi-asserted-by":"publisher","first-page":"1630","DOI":"10.1101\/gad.260003","volume":"17","author":"JM Ng","year":"2003","unstructured":"Ng JM, Vermeulen W, van der Horst GT, Bergink S, Sugasawa K, Vrieling H et al (2003) A novel regulation mechanism of DNA repair by damage-induced and RAD23-dependent stabilization of xeroderma pigmentosum group C protein. Genes Dev 17(13):1630\u20131645","journal-title":"Genes Dev"},{"issue":"11","key":"10_CR53","doi-asserted-by":"publisher","first-page":"1619","DOI":"10.1016\/j.bbamcr.2007.07.012","volume":"1773","author":"A Ferro","year":"2007","unstructured":"Ferro A, Carvalho AL, Teixeira-Castro A, Almeida C, Tome RJ, Cortes L et al (2007) NEDD8: a new ataxin-3 interactor. Biochim Biophys Acta 1773(11):1619\u20131627","journal-title":"Biochim Biophys Acta"},{"issue":"1","key":"10_CR54","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1080\/19768354.2012.738611","volume":"17","author":"NR Jung","year":"2013","unstructured":"Jung NR, Lee DH (2013) SUMO-1 promotes degradation of the polyglutamine disease protein ataxin-3. Anim Cells Syst 17(1):7\u201314","journal-title":"Anim Cells Syst"},{"issue":"8","key":"10_CR55","doi-asserted-by":"publisher","first-page":"1066","DOI":"10.15252\/embj.201695151","volume":"36","author":"A Pfeiffer","year":"2017","unstructured":"Pfeiffer A, Luijsterburg MS, Acs K, Wiegant WW, Helfricht A, Herzog LK et al (2017) Ataxin-3 consolidates the MDC1-dependent DNA double-strand break response by counteracting the SUMO-targeted ubiquitin ligase RNF4. EMBO J 36(8):1066\u20131083","journal-title":"EMBO J"},{"issue":"7","key":"10_CR56","doi-asserted-by":"publisher","first-page":"1015","DOI":"10.4161\/cc.24332","volume":"12","author":"CM Guzzo","year":"2013","unstructured":"Guzzo CM, Matunis MJ (2013) Expanding SUMO and ubiquitin-mediated signaling through hybrid SUMO-ubiquitin chains and their receptors. Cell Cycle 12(7):1015\u20131017","journal-title":"Cell Cycle"},{"issue":"253","key":"10_CR57","doi-asserted-by":"publisher","first-page":"ra88","DOI":"10.1126\/scisignal.2003485","volume":"5","author":"CM Guzzo","year":"2012","unstructured":"Guzzo CM, Berndsen CE, Zhu J, Gupta V, Datta A, Greenberg RA et al (2012) RNF4-dependent hybrid SUMO-ubiquitin chains are signals for RAP80 and thereby mediate the recruitment of BRCA1 to sites of DNA damage. Sci Signal 5(253):ra88","journal-title":"Sci Signal"},{"issue":"22","key":"10_CR58","doi-asserted-by":"publisher","first-page":"2689","DOI":"10.1093\/hmg\/11.22.2689","volume":"11","author":"LG Verhoef","year":"2002","unstructured":"Verhoef LG, Lindsten K, Masucci MG, Dantuma NP (2002) Aggregate formation inhibits proteasomal degradation of polyglutamine proteins. Hum Mol Genet 11(22):2689\u20132700","journal-title":"Hum Mol Genet"},{"issue":"2","key":"10_CR59","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1038\/nrm3741","volume":"15","author":"S Bhattacharyya","year":"2014","unstructured":"Bhattacharyya S, Yu H, Mim C, Matouschek A (2014) Regulated protein turnover: snapshots of the proteasome in action. Nat Rev Mol Cell Biol 15(2):122\u2013133","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"7","key":"10_CR60","doi-asserted-by":"publisher","first-page":"458","DOI":"10.1038\/nrm2708","volume":"10","author":"H Nakatogawa","year":"2009","unstructured":"Nakatogawa H, Suzuki K, Kamada Y, Ohsumi Y (2009) Dynamics and diversity in autophagy mechanisms: lessons from yeast. Nat Rev Mol Cell Biol 10(7):458\u2013467","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"6","key":"10_CR61","doi-asserted-by":"publisher","first-page":"1239","DOI":"10.1083\/jcb.146.6.1239","volume":"146","author":"R Garcia-Mata","year":"1999","unstructured":"Garcia-Mata R, Bebok Z, Sorscher EJ, Sztul ES (1999) Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera. J Cell Biol 146(6):1239\u20131254","journal-title":"J Cell Biol"},{"issue":"9071","key":"10_CR62","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1016\/S0140-6736(97)02073-4","volume":"350","author":"RW Carrell","year":"1997","unstructured":"Carrell RW, Lomas DA (1997) Conformational disease. Lancet 350(9071):134\u2013138","journal-title":"Lancet"},{"key":"10_CR63","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1016\/j.arr.2014.07.002","volume":"18","author":"JM Hyttinen","year":"2014","unstructured":"Hyttinen JM, Amadio M, Viiri J, Pascale A, Salminen A, Kaarniranta K (2014) Clearance of misfolded and aggregated proteins by aggrephagy and implications for aggregation diseases. Ageing Res Rev 18:16\u201328","journal-title":"Ageing Res Rev"},{"key":"10_CR64","doi-asserted-by":"crossref","unstructured":"Michalik A, Van Broeckhoven C (2003) Pathogenesis of polyglutamine disorders: aggregation revisited. Hum Mol Genet 12(Spec No. 2):R173\u2013186","DOI":"10.1093\/hmg\/ddg295"},{"issue":"8","key":"10_CR65","doi-asserted-by":"publisher","first-page":"766","DOI":"10.1038\/ncb0805-766","volume":"7","author":"B Meusser","year":"2005","unstructured":"Meusser B, Hirsch C, Jarosch E, Sommer T (2005) ERAD: the long road to destruction. Nat Cell Biol 7(8):766\u2013772","journal-title":"Nat Cell Biol"},{"issue":"40","key":"10_CR66","doi-asserted-by":"publisher","first-page":"14296","DOI":"10.1073\/pnas.0505014102","volume":"102","author":"BN Lilley","year":"2005","unstructured":"Lilley BN, Ploegh HL (2005) Multiprotein complexes that link dislocation, ubiquitination, and extraction of misfolded proteins from the endoplasmic reticulum membrane. Proc Natl Acad Sci U S A 102(40):14296\u201314301","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"40","key":"10_CR67","doi-asserted-by":"publisher","first-page":"14132","DOI":"10.1073\/pnas.0505006102","volume":"102","author":"Y Ye","year":"2005","unstructured":"Ye Y, Shibata Y, Kikkert M, van Voorden S, Wiertz E, Rapoport TA (2005) Inaugural article: recruitment of the p97 ATPase and ubiquitin ligases to the site of retrotranslocation at the endoplasmic reticulum membrane. Proc Natl Acad Sci U S A 102(40):14132\u201314138","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10_CR68","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1146\/annurev.biochem.78.081507.101607","volume":"78","author":"D Finley","year":"2009","unstructured":"Finley D (2009) Recognition and processing of ubiquitin-protein conjugates by the proteasome. Annu Rev Biochem 78:477\u2013513","journal-title":"Annu Rev Biochem"},{"issue":"7","key":"10_CR69","doi-asserted-by":"publisher","first-page":"963","DOI":"10.1083\/jcb.200605100","volume":"174","author":"Q Wang","year":"2006","unstructured":"Wang Q, Li L, Ye Y (2006) Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3. J Cell Biol 174(7):963\u2013971","journal-title":"J Cell Biol"},{"issue":"6","key":"10_CR70","doi-asserted-by":"publisher","first-page":"4892","DOI":"10.1074\/jbc.M110.158030","volume":"286","author":"DP LaLonde","year":"2011","unstructured":"LaLonde DP, Bretscher A (2011) The UBX protein SAKS1 negatively regulates endoplasmic reticulum-associated degradation and p97-dependent degradation. J Biol Chem 286(6):4892\u20134901","journal-title":"J Biol Chem"},{"issue":"12","key":"10_CR71","doi-asserted-by":"publisher","first-page":"7445","DOI":"10.1074\/jbc.M708347200","volume":"283","author":"Q Wang","year":"2008","unstructured":"Wang Q, Li L, Ye Y (2008) Inhibition of p97-dependent protein degradation by Eeyarestatin I. J Biol Chem 283(12):7445\u20137454","journal-title":"J Biol Chem"},{"issue":"16","key":"10_CR72","doi-asserted-by":"publisher","first-page":"2409","DOI":"10.1093\/hmg\/ddl164","volume":"15","author":"X Zhong","year":"2006","unstructured":"Zhong X, Pittman RN (2006) Ataxin-3 binds VCP\/p97 and regulates retrotranslocation of ERAD substrates. Hum Mol Genet 15(16):2409\u20132420","journal-title":"Hum Mol Genet"},{"issue":"22","key":"10_CR73","doi-asserted-by":"publisher","first-page":"4268","DOI":"10.1093\/hmg\/ddp380","volume":"18","author":"Z Ying","year":"2009","unstructured":"Ying Z, Wang H, Fan H, Zhu X, Zhou J, Fei E et al (2009) Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation. Hum Mol Genet 18(22):4268\u20134281","journal-title":"Hum Mol Genet"},{"issue":"3","key":"10_CR74","doi-asserted-by":"publisher","first-page":"e1001038","DOI":"10.1371\/journal.pbio.1001038","volume":"9","author":"YC Tsai","year":"2011","unstructured":"Tsai YC, Weissman AM (2011) Ubiquitylation in ERAD: reversing to go forward? PLoS Biol 9(3):e1001038","journal-title":"PLoS Biol"},{"issue":"9","key":"10_CR75","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1016\/j.tcb.2012.06.003","volume":"22","author":"NP Dantuma","year":"2012","unstructured":"Dantuma NP, Hoppe T (2012) Growing sphere of influence: Cdc48\/p97 orchestrates ubiquitin-dependent extraction from chromatin. Trends Cell Biol 22(9):483\u2013491","journal-title":"Trends Cell Biol"},{"issue":"2","key":"10_CR76","doi-asserted-by":"publisher","first-page":"182","DOI":"10.1016\/j.molcel.2017.10.028","volume":"69","author":"J Boom van den","year":"2018","unstructured":"van den Boom J, Meyer H (2018) VCP\/p97-mediated unfolding as a principle in protein homeostasis and signaling. Mol Cell 69(2):182\u2013194","journal-title":"Mol Cell"},{"issue":"4","key":"10_CR77","doi-asserted-by":"publisher","first-page":"732","DOI":"10.1016\/j.jmb.2009.09.050","volume":"394","author":"A Beskow","year":"2009","unstructured":"Beskow A, Grimberg KB, Bott LC, Salomons FA, Dantuma NP, Young P (2009) A conserved unfoldase activity for the p97 AAA-ATPase in proteasomal degradation. J Mol Biol 394(4):732\u2013746","journal-title":"J Mol Biol"},{"key":"10_CR78","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1038\/s42003-019-0283-z","volume":"2","author":"MM Olszewski","year":"2019","unstructured":"Olszewski MM, Williams C, Dong KC, Martin A (2019) The Cdc48 unfoldase prepares well-folded protein substrates for degradation by the 26S proteasome. Commun Biol 2:29","journal-title":"Commun Biol"},{"issue":"7349","key":"10_CR79","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1038\/nature09966","volume":"474","author":"DM Wenzel","year":"2011","unstructured":"Wenzel DM, Lissounov A, Brzovic PS, Klevit RE (2011) UBCH7 reactivity profile reveals parkin and HHARI to be RING\/HECT hybrids. Nature (London) 474(7349):105\u2013108","journal-title":"Nature (London)"},{"issue":"6","key":"10_CR80","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1016\/S0896-6273(03)00143-0","volume":"37","author":"Y Yang","year":"2003","unstructured":"Yang Y, Nishimura I, Imai Y, Takahashi R, Lu B (2003) Parkin suppresses dopaminergic neuron-selective neurotoxicity induced by pael-R in Drosophila. Neuron 37(6):911\u2013924","journal-title":"Neuron"},{"issue":"1","key":"10_CR81","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1093\/hmg\/ddl439","volume":"16","author":"Y Kitao","year":"2007","unstructured":"Kitao Y, Imai Y, Ozawa K, Kataoka A, Ikeda T, Soda M et al (2007) Pael receptor induces death of dopaminergic neurons in the substantia nigra via endoplasmic reticulum stress and dopamine toxicity, which is enhanced under condition of parkin inactivation. Hum Mol Genet 16(1):50\u201360","journal-title":"Hum Mol Genet"},{"issue":"2","key":"10_CR82","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1016\/j.neuron.2014.12.007","volume":"85","author":"AM Pickrell","year":"2015","unstructured":"Pickrell AM, Youle RJ (2015) The roles of PINK1, parkin, and mitochondrial fidelity in Parkinson\u2019s disease. Neuron 85(2):257\u2013273","journal-title":"Neuron"},{"issue":"4","key":"10_CR83","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1016\/j.ceb.2011.05.007","volume":"23","author":"M Karbowski","year":"2011","unstructured":"Karbowski M, Youle RJ (2011) Regulating mitochondrial outer membrane proteins by ubiquitination and proteasomal degradation. Curr Opin Cell Biol 23(4):476\u2013482","journal-title":"Curr Opin Cell Biol"},{"issue":"2","key":"10_CR84","doi-asserted-by":"publisher","first-page":"119","DOI":"10.1038\/ncb2012","volume":"12","author":"S Geisler","year":"2010","unstructured":"Geisler S, Holmstrom KM, Skujat D, Fiesel FC, Rothfuss OC, Kahle PJ et al (2010) PINK1\/Parkin-mediated mitophagy is dependent on VDAC1 and p62\/SQSTM1. Nat Cell Biol 12(2):119\u2013131","journal-title":"Nat Cell Biol"},{"issue":"2","key":"10_CR85","doi-asserted-by":"publisher","first-page":"308","DOI":"10.1016\/j.molcel.2017.09.005","volume":"68","author":"S Jin","year":"2017","unstructured":"Jin S, Tian S, Luo M, Xie W, Liu T, Duan T et al (2017) Tetherin suppresses type I interferon signaling by targeting MAVS for NDP52-mediated selective autophagic degradation in human cells. Mol Cell 68(2):308\u2013322","journal-title":"Mol Cell"},{"issue":"1","key":"10_CR86","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1074\/jbc.M111.288449","volume":"287","author":"TM Durcan","year":"2012","unstructured":"Durcan TM, Kontogiannea M, Bedard N, Wing SS, Fon EA (2012) Ataxin-3 deubiquitination is coupled to Parkin ubiquitination via E2 ubiquitin-conjugating enzyme. J Biol Chem 287(1):531\u2013541","journal-title":"J Biol Chem"},{"issue":"7652","key":"10_CR87","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1038\/nature22078","volume":"545","author":"A Ashkenazi","year":"2017","unstructured":"Ashkenazi A, Bento CF, Ricketts T, Vicinanza M, Siddiqi F, Pavel M et al (2017) Polyglutamine tracts regulate beclin 1-dependent autophagy. Nature (London) 545(7652):108\u2013111","journal-title":"Nature (London)"},{"issue":"7095","key":"10_CR88","doi-asserted-by":"publisher","first-page":"885","DOI":"10.1038\/nature04724","volume":"441","author":"T Hara","year":"2006","unstructured":"Hara T, Nakamura K, Matsui M, Yamamoto A, Nakahara Y, Suzuki-Migishima R et al (2006) Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice. Nature (London) 441(7095):885\u2013889","journal-title":"Nature (London)"},{"issue":"7095","key":"10_CR89","doi-asserted-by":"publisher","first-page":"880","DOI":"10.1038\/nature04723","volume":"441","author":"M Komatsu","year":"2006","unstructured":"Komatsu M, Waguri S, Chiba T, Murata S, Iwata J, Tanida I et al (2006) Loss of autophagy in the central nervous system causes neurodegeneration in mice. Nature (London) 441(7095):880\u2013884","journal-title":"Nature (London)"},{"issue":"6","key":"10_CR90","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1038\/nrn3961","volume":"16","author":"FM Menzies","year":"2015","unstructured":"Menzies FM, Fleming A, Rubinsztein DC (2015) Compromised autophagy and neurodegenerative diseases. Nat Rev Neurosci 16(6):345\u2013357","journal-title":"Nat Rev Neurosci"},{"issue":"12","key":"10_CR91","doi-asserted-by":"publisher","first-page":"4330","DOI":"10.1073\/pnas.0407252102","volume":"102","author":"BG Burnett","year":"2005","unstructured":"Burnett BG, Pittman RN (2005) The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation. Proc Natl Acad Sci U S A 102(12):4330\u20134335","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10_CR92","doi-asserted-by":"publisher","first-page":"427","DOI":"10.1016\/S0896-6273(03)00606-8","volume":"40","author":"A Ciechanover","year":"2003","unstructured":"Ciechanover A, Brundin P (2003) The ubiquitin proteasome system in neuordegenerative diseases: sometimes the chicken, sometimes the egg. Neuron 40:427\u2013446","journal-title":"Neuron"},{"issue":"6","key":"10_CR93","doi-asserted-by":"publisher","first-page":"727","DOI":"10.1016\/S0092-8674(03)00939-5","volume":"115","author":"Y Kawaguchi","year":"2003","unstructured":"Kawaguchi Y, Kovacs JJ, McLaurin A, Vance JM, Ito A, Yao TP (2003) The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress. Cell 115(6):727\u2013738","journal-title":"Cell"},{"issue":"14","key":"10_CR94","doi-asserted-by":"publisher","first-page":"3357","DOI":"10.1038\/sj.emboj.7601210","volume":"25","author":"C Boyault","year":"2006","unstructured":"Boyault C, Gilquin B, Zhang Y, Rybin V, Garman E, Meyer-Klaucke W et al (2006) HDAC6-p97\/VCP controlled polyubiquitin chain turnover. EMBO J 25(14):3357\u20133366","journal-title":"EMBO J"},{"issue":"4","key":"10_CR95","doi-asserted-by":"publisher","first-page":"2317","DOI":"10.1074\/jbc.M111.273730","volume":"287","author":"H Ouyang","year":"2012","unstructured":"Ouyang H, Ali YO, Ravichandran M, Dong A, Qiu W, MacKenzie F et al (2012) Protein aggregates are recruited to aggresome by histone deacetylase 6 via unanchored ubiquitin C termini. J Biol Chem 287(4):2317\u20132327","journal-title":"J Biol Chem"},{"issue":"6","key":"10_CR96","doi-asserted-by":"publisher","first-page":"991","DOI":"10.1093\/hmg\/7.6.991","volume":"7","author":"D Tait","year":"1998","unstructured":"Tait D, Riccio M, Sittler A, Scherzinger E, Santi S, Ognibene A et al (1998) Ataxin-3 is transported into the nucleus and associates with the nuclear matrix. Hum Mol Genet 7(6):991\u2013997","journal-title":"Hum Mol Genet"},{"issue":"28","key":"10_CR97","doi-asserted-by":"publisher","first-page":"7418","DOI":"10.1523\/JNEUROSCI.4540-06.2007","volume":"27","author":"U Bichelmeier","year":"2007","unstructured":"Bichelmeier U, Schmidt T, Hubener J, Boy J, Ruttiger L, Habig K et al (2007) Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence. J Neurosci 27(28):7418\u20137428","journal-title":"J Neurosci"},{"issue":"2","key":"10_CR98","doi-asserted-by":"publisher","first-page":"280","DOI":"10.1016\/j.nbd.2009.07.020","volume":"36","author":"PM Antony","year":"2009","unstructured":"Antony PM, Mantele S, Mollenkopf P, Boy J, Kehlenbach RH, Riess O et al (2009) Identification and functional dissection of localization signals within ataxin-3. Neurobiol Dis 36(2):280\u2013292","journal-title":"Neurobiol Dis"},{"issue":"2","key":"10_CR99","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1093\/hmg\/ddp482","volume":"19","author":"CP Reina","year":"2010","unstructured":"Reina CP, Zhong X, Pittman RN (2010) Proteotoxic stress increases nuclear localization of ataxin-3. Hum Mol Genet 19(2):235\u2013249","journal-title":"Hum Mol Genet"},{"issue":"1","key":"10_CR100","doi-asserted-by":"publisher","first-page":"e1004749","DOI":"10.1371\/journal.pgen.1004749","volume":"11","author":"A Chatterjee","year":"2015","unstructured":"Chatterjee A, Saha S, Chakraborty A, Silva-Fernandes A, Mandal SM, Neves-Carvalho A et al (2015) The role of the mammalian DNA end-processing enzyme polynucleotide kinase 3\u2019-phosphatase in spinocerebellar ataxia type 3 pathogenesis. PLoS Genet 11(1):e1004749","journal-title":"PLoS Genet"},{"issue":"5","key":"10_CR101","doi-asserted-by":"publisher","first-page":"262","DOI":"10.1016\/j.tibs.2011.01.006","volume":"36","author":"M Weinfeld","year":"2011","unstructured":"Weinfeld M, Mani RS, Abdou I, Aceytuno RD, Glover JN (2011) Tidying up loose ends: the role of polynucleotide kinase\/phosphatase in DNA strand break repair. Trends Biochem Sci 36(5):262\u2013271","journal-title":"Trends Biochem Sci"},{"issue":"1","key":"10_CR102","doi-asserted-by":"publisher","first-page":"e1004834","DOI":"10.1371\/journal.pgen.1004834","volume":"11","author":"R Gao","year":"2015","unstructured":"Gao R, Liu Y, Silva-Fernandes A, Fang X, Paulucci-Holthauzen A, Chatterjee A et al (2015) Inactivation of PNKP by mutant ATXN3 triggers apoptosis by activating the DNA damage-response pathway in SCA3. PLoS Genet 11(1):e1004834","journal-title":"PLoS Genet"},{"issue":"3","key":"10_CR103","doi-asserted-by":"publisher","first-page":"708","DOI":"10.1016\/j.nbd.2006.06.005","volume":"23","author":"S Bergink","year":"2006","unstructured":"Bergink S, Severijnen LA, Wijgers N, Sugasawa K, Yousaf H, Kros JM et al (2006) The DNA repair-ubiquitin-associated HR23 proteins are constituents of neuronal inclusions in specific neurodegenerative disorders without hampering DNA repair. Neurobiol Dis 23(3):708\u2013716","journal-title":"Neurobiol Dis"},{"issue":"6","key":"10_CR104","doi-asserted-by":"publisher","first-page":"2923","DOI":"10.1096\/fj.201700801RR","volume":"32","author":"H Yang","year":"2018","unstructured":"Yang H, Yue HW, He WT, Hong JY, Jiang LL, Hu HY (2018) PolyQ-expanded huntingtin and ataxin-3 sequester ubiquitin adaptors hHR23B and UBQLN2 into aggregates via conjugated ubiquitin. FASEB J 32(6):2923\u20132933","journal-title":"FASEB J"},{"issue":"1","key":"10_CR105","doi-asserted-by":"publisher","first-page":"6","DOI":"10.15252\/embj.201592595","volume":"35","author":"NP Dantuma","year":"2016","unstructured":"Dantuma NP, van Attikum H (2016) Spatiotemporal regulation of posttranslational modifications in the DNA damage response. EMBO J 35(1):6\u201323","journal-title":"EMBO J"},{"issue":"1","key":"10_CR106","doi-asserted-by":"publisher","first-page":"e54214","DOI":"10.1371\/journal.pone.0054214","volume":"8","author":"YF Zhou","year":"2013","unstructured":"Zhou YF, Liao SS, Luo YY, Tang JG, Wang JL, Lei LF et al (2013) SUMO-1 modification on K166 of polyQ-expanded ataxin-3 strengthens its stability and increases its cytotoxicity. PLoS One 8(1):e54214","journal-title":"PLoS One"},{"issue":"8","key":"10_CR107","doi-asserted-by":"publisher","first-page":"4532","DOI":"10.1093\/nar\/gkx095","volume":"45","author":"Y Tu","year":"2017","unstructured":"Tu Y, Liu H, Zhu X, Shen H, Ma X, Wang F et al (2017) Ataxin-3 promotes genome integrity by stabilizing Chk1. Nucleic Acids Res 45(8):4532\u20134549","journal-title":"Nucleic Acids Res"},{"issue":"6","key":"10_CR108","doi-asserted-by":"publisher","first-page":"2630","DOI":"10.1158\/0008-5472.CAN-08-3382","volume":"69","author":"V Leung-Pineda","year":"2009","unstructured":"Leung-Pineda V, Huh J, Piwnica-Worms H (2009) DDB1 targets Chk1 to the Cul4 E3 ligase complex in normal cycling cells and in cells experiencing replication stress. Cancer Res 69(6):2630\u20132637","journal-title":"Cancer Res"},{"issue":"5","key":"10_CR109","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1016\/j.molcel.2005.07.019","volume":"19","author":"YW Zhang","year":"2005","unstructured":"Zhang YW, Otterness DM, Chiang GG, Xie W, Liu YC, Mercurio F et al (2005) Genotoxic stress targets human Chk1 for degradation by the ubiquitin-proteasome pathway. Mol Cell 19(5):607\u2013618","journal-title":"Mol Cell"},{"issue":"6","key":"10_CR110","doi-asserted-by":"publisher","first-page":"642","DOI":"10.1016\/S1470-2045(12)70132-8","volume":"13","author":"J Ji","year":"2012","unstructured":"Ji J, Sundquist K, Sundquist J (2012) Cancer incidence in patients with polyglutamine diseases: a population-based study in Sweden. Lancet Oncol 13(6):642\u2013648","journal-title":"Lancet Oncol"},{"issue":"47","key":"10_CR111","doi-asserted-by":"publisher","first-page":"45004","DOI":"10.1074\/jbc.M205259200","volume":"277","author":"F Li","year":"2002","unstructured":"Li F, Macfarlan T, Pittman RN, Chakravarti D (2002) Ataxin-3 Is a histone-binding protein with two independent transcriptional corepressor activities. J Biol Chem 277(47):45004\u201345012","journal-title":"J Biol Chem"},{"issue":"10","key":"10_CR112","doi-asserted-by":"publisher","first-page":"562","DOI":"10.1016\/j.tig.2006.07.010","volume":"22","author":"D Helmlinger","year":"2006","unstructured":"Helmlinger D, Tora L, Devys D (2006) Transcriptional alterations and chromatin remodeling in polyglutamine diseases. Trends Genet 22(10):562\u2013570","journal-title":"Trends Genet"},{"issue":"2","key":"10_CR113","doi-asserted-by":"publisher","first-page":"220","DOI":"10.1016\/j.molcel.2015.09.017","volume":"60","author":"H Zhang","year":"2015","unstructured":"Zhang H, Elbaum-Garfinkle S, Langdon EM, Taylor N, Occhipinti P, Bridges AA et al (2015) RNA controls PolyQ protein phase transitions. Mol Cell 60(2):220\u2013230","journal-title":"Mol Cell"},{"issue":"3","key":"10_CR114","doi-asserted-by":"publisher","first-page":"734","DOI":"10.1016\/j.bbrc.2007.08.062","volume":"362","author":"I Schmitt","year":"2007","unstructured":"Schmitt I, Linden M, Khazneh H, Evert BO, Breuer P, Klockgether T et al (2007) Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination. Biochem Biophys Res Commun 362(3):734\u2013739","journal-title":"Biochem Biophys Res Commun"},{"issue":"12","key":"10_CR115","doi-asserted-by":"publisher","first-page":"2380","DOI":"10.1093\/hmg\/ddq111","volume":"19","author":"S Alves","year":"2010","unstructured":"Alves S, Nascimento-Ferreira I, Dufour N, Hassig R, Auregan G, Nobrega C et al (2010) Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3? Hum Mol Genet 19(12):2380\u20132394","journal-title":"Hum Mol Genet"},{"issue":"4","key":"10_CR116","doi-asserted-by":"publisher","first-page":"e18512","DOI":"10.1371\/journal.pone.0018512","volume":"6","author":"AJ Rodrigues","year":"2011","unstructured":"Rodrigues AJ, Neves-Carvalho A, Teixeira-Castro A, Rokka A, Corthals G, Logarinho E et al (2011) Absence of ataxin-3 leads to enhanced stress response in C. elegans. PLoS One 6(4):e18512","journal-title":"PLoS One"},{"issue":"22","key":"10_CR117","doi-asserted-by":"publisher","first-page":"4282","DOI":"10.1093\/hmg\/ddp381","volume":"18","author":"J Boy","year":"2009","unstructured":"Boy J, Schmidt T, Wolburg H, Mack A, Nuber S, Bottcher M et al (2009) Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3. Hum Mol Genet 18(22):4282\u20134295","journal-title":"Hum Mol Genet"},{"issue":"1","key":"10_CR118","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1016\/S0092-8674(00)80623-6","volume":"101","author":"A Yamamoto","year":"2000","unstructured":"Yamamoto A, Lucas JJ, Hen R (2000) Reversal of neuropathology and motor dysfunction in a conditional model of Huntington\u2019s disease. Cell 101(1):57\u201366","journal-title":"Cell"},{"issue":"9","key":"10_CR119","doi-asserted-by":"publisher","first-page":"1463","DOI":"10.1093\/hmg\/ddy437","volume":"28","author":"J Schmidt","year":"2019","unstructured":"Schmidt J, Mayer AK, Bakula D, Freude J, Weber JJ, Weiss A et al (2019) Vulnerability of frontal brain neurons for the toxicity of expanded ataxin-3. Hum Mol Genet 28(9):1463\u20131473","journal-title":"Hum Mol Genet"},{"issue":"10","key":"10_CR120","doi-asserted-by":"publisher","first-page":"609","DOI":"10.1016\/j.molmed.2015.07.006","volume":"21","author":"LE Dow","year":"2015","unstructured":"Dow LE (2015) modeling disease in vivo with CRISPR\/Cas9. Trends Mol Med 21(10):609\u2013621","journal-title":"Trends Mol Med"},{"key":"10_CR121","doi-asserted-by":"crossref","unstructured":"Kolli N, Lu M, Maiti P, Rossignol J, Dunbar GL (2017) CRISPR-Cas9 mediated gene-silencing of the mutant huntingtin gene in an in vitro model of Huntington\u2019s disease. Int J Mol Sci 18(4)","DOI":"10.3390\/ijms18040754"},{"issue":"20","key":"10_CR122","first-page":"4566","volume":"25","author":"JW Shin","year":"2016","unstructured":"Shin JW, Kim KH, Chao MJ, Atwal RS, Gillis T, MacDonald ME et al (2016) Permanent inactivation of Huntington\u2019s disease mutation by personalized allele-specific CRISPR\/Cas9. Hum Mol Genet 25(20):4566\u20134576","journal-title":"Hum Mol Genet"},{"issue":"1","key":"10_CR123","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.ymthe.2016.11.010","volume":"25","author":"AM Monteys","year":"2017","unstructured":"Monteys AM, Ebanks SA, Keiser MS, Davidson BL (2017) CRISPR\/Cas9 editing of the mutant Huntingtin allele in vitro and in vivo. Mol Ther 25(1):12\u201323","journal-title":"Mol Ther"},{"issue":"7","key":"10_CR124","doi-asserted-by":"publisher","first-page":"2719","DOI":"10.1172\/JCI92087","volume":"127","author":"S Yang","year":"2017","unstructured":"Yang S, Chang R, Yang H, Zhao T, Hong Y, Kong HE et al (2017) CRISPR\/Cas9-mediated gene editing ameliorates neurotoxicity in mouse model of Huntington\u2019s disease. J Clin Invest 127(7):2719\u20132724","journal-title":"J Clin Invest"},{"issue":"11","key":"10_CR125","doi-asserted-by":"publisher","first-page":"756","DOI":"10.1089\/scd.2017.0209","volume":"27","author":"S Ouyang","year":"2018","unstructured":"Ouyang S, Xie Y, Xiong Z, Yang Y, Xian Y, Ou Z et al (2018) CRISPR\/Cas9-targeted deletion of polyglutamine in spinocerebellar ataxia type 3-derived induced pluripotent stem cells. Stem Cells Dev 27(11):756\u2013770","journal-title":"Stem Cells Dev"},{"issue":"10","key":"10_CR126","doi-asserted-by":"publisher","first-page":"e3341","DOI":"10.1371\/journal.pone.0003341","volume":"3","author":"S Alves","year":"2008","unstructured":"Alves S, Nascimento-Ferreira I, Auregan G, Hassig R, Dufour N, Brouillet E et al (2008) Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease. PLoS One 3(10):e3341","journal-title":"PLoS One"},{"issue":"1","key":"10_CR127","doi-asserted-by":"publisher","first-page":"e52396","DOI":"10.1371\/journal.pone.0052396","volume":"8","author":"C N\u00f3brega","year":"2013","unstructured":"N\u00f3brega C, Nascimento-Ferreira I, Onofre I, Albuquerque D, Hirai H, D\u00e9glon N et al (2013) Silencing mutant ataxin-3 rescues motor deficits and neuropathology in machado-joseph disease transgenic mice. PLoS One 8(1):e52396","journal-title":"PLoS One"},{"key":"10_CR128","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1016\/j.biomaterials.2015.12.021","volume":"82","author":"M Concei\u00e7\u00e3o","year":"2016","unstructured":"Concei\u00e7\u00e3o M, Mendon\u00e7a L, N\u00f3brega C, Gomes C, Costa P, Hirai H et al (2016) Intravenous administration of brain-targeted stable nucleic acid lipid particles alleviates Machado-Joseph disease neurological phenotype. Biomaterials 82:124\u2013137","journal-title":"Biomaterials"},{"key":"10_CR129","doi-asserted-by":"crossref","first-page":"175628641877693","DOI":"10.1177\/1756286418776932","volume":"11","author":"CD Wurster","year":"2018","unstructured":"Wurster CD, Ludolph AC (2018) Antisense oligonucleotides in neurological disorders. Ther Adv Neurol Disord 11:1756286418776932","journal-title":"Ther Adv Neurol Disord"},{"issue":"1","key":"10_CR130","doi-asserted-by":"publisher","first-page":"280","DOI":"10.1073\/pnas.75.1.280","volume":"75","author":"PC Zamecnik","year":"1978","unstructured":"Zamecnik PC, Stephenson ML (1978) Inhibition of Rous sarcoma virus replication and cell transformation by a specific oligodeoxynucleotide. Proc Natl Acad Sci U S A 75(1):280\u2013284","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"10","key":"10_CR131","doi-asserted-by":"publisher","first-page":"837","DOI":"10.1016\/S1474-4422(17)30280-6","volume":"16","author":"EJ Wild","year":"2017","unstructured":"Wild EJ, Tabrizi SJ (2017) Therapies targeting DNA and RNA in Huntington\u2019s disease. Lancet Neurol 16(10):837\u2013847","journal-title":"Lancet Neurol"},{"key":"10_CR132","first-page":"200","volume":"7","author":"LR Moore","year":"2017","unstructured":"Moore LR, Rajpal G, Dillingham IT, Qutob M, Blumenstein KG, Gattis D et al (2017) Evaluation of antisense oligonucleotides targeting ATXN3 in SCA3 mouse models. Mol Ther 7:200\u2013210","journal-title":"Mol Ther"},{"key":"10_CR133","first-page":"232","volume":"8","author":"LJA Toonen","year":"2017","unstructured":"Toonen LJA, Rigo F, van Attikum H, van Roon-Mom WMC (2017) Antisense oligonucleotide-mediated removal of the polyglutamine repeat in spinocerebellar ataxia type 3 mice. Mol Ther 8:232\u2013242","journal-title":"Mol Ther"},{"key":"10_CR134","doi-asserted-by":"publisher","first-page":"35200","DOI":"10.1038\/srep35200","volume":"6","author":"LJA Toonen","year":"2016","unstructured":"Toonen LJA, Schmidt I, Luijsterburg MS, van Attikum H, van Roon-Mom WMC (2016) Antisense oligonucleotide-mediated exon skipping as a strategy to reduce proteolytic cleavage of ataxin-3. Sci Rep 6:35200","journal-title":"Sci Rep"},{"issue":"5","key":"10_CR135","doi-asserted-by":"publisher","first-page":"478","DOI":"10.1038\/nbt.1539","volume":"27","author":"J Hu","year":"2009","unstructured":"Hu J, Matsui M, Gagnon KT, Schwartz JC, Gabillet S, Arar K et al (2009) Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs. Nat Biotechnol 27(5):478\u2013484","journal-title":"Nat Biotechnol"},{"issue":"Pt 5","key":"10_CR136","doi-asserted-by":"publisher","first-page":"1400","DOI":"10.1093\/brain\/awr047","volume":"134","author":"I Nascimento-Ferreira","year":"2011","unstructured":"Nascimento-Ferreira I, Santos-Ferreira T, Sousa-Ferreira L, Auregan G, Onofre I, Alves S et al (2011) Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease. Brain 134(Pt 5):1400\u20131415","journal-title":"Brain"},{"issue":"Pt 7","key":"10_CR137","doi-asserted-by":"publisher","first-page":"2173","DOI":"10.1093\/brain\/awt144","volume":"136","author":"I Nascimento-Ferreira","year":"2013","unstructured":"Nascimento-Ferreira I, Nobrega C, Vasconcelos-Ferreira A, Onofre I, Albuquerque D, Aveleira C et al (2013) Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease. Brain J Neurol 136(Pt 7):2173\u20132188","journal-title":"Brain J Neurol"},{"issue":"7436","key":"10_CR138","doi-asserted-by":"publisher","first-page":"201","DOI":"10.1038\/nature11866","volume":"494","author":"S Shoji-Kawata","year":"2013","unstructured":"Shoji-Kawata S, Sumpter R, Leveno M, Campbell GR, Zou Z, Kinch L et al (2013) Identification of a candidate therapeutic autophagy-inducing peptide. Nature (London) 494(7436):201\u2013206","journal-title":"Nature (London)"},{"issue":"5521","key":"10_CR139","doi-asserted-by":"publisher","first-page":"1552","DOI":"10.1126\/science.292.5521.1552","volume":"292","author":"NF Bence","year":"2001","unstructured":"Bence NF, Sampat RM, Kopito RR (2001) Impairment of the ubiquitin-proteasome system by protein aggregation. Science 292(5521):1552\u20131555","journal-title":"Science"},{"issue":"10","key":"10_CR140","doi-asserted-by":"publisher","first-page":"3675","DOI":"10.1523\/JNEUROSCI.5673-09.2010","volume":"30","author":"Z Ortega","year":"2010","unstructured":"Ortega Z, Diaz-Hernandez M, Maynard CJ, Hernandez F, Dantuma NP, Lucas JJ (2010) Acute polyglutamine expression in inducible mouse model unravels ubiquitin\/proteasome system impairment and permanent recovery attributable to aggregate formation. J Neurosci 30(10):3675\u20133688","journal-title":"J Neurosci"},{"key":"10_CR141","first-page":"20","volume":"20","author":"AB Bowman","year":"2005","unstructured":"Bowman AB, Yoo SY, Dantuma NP, Zoghbi HY (2005) Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation. Hum Mol Genet 20:20","journal-title":"Hum Mol Genet"},{"issue":"33","key":"10_CR142","doi-asserted-by":"publisher","first-page":"13986","DOI":"10.1073\/pnas.0906463106","volume":"106","author":"CJ Maynard","year":"2009","unstructured":"Maynard CJ, Bottcher C, Ortega Z, Smith R, Florea BI, Diaz-Hernandez M et al (2009) Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin\/proteasome system impairment. Proc Natl Acad Sci U S A 106(33):13986\u201313991","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"5","key":"10_CR143","doi-asserted-by":"publisher","first-page":"898","DOI":"10.1093\/hmg\/ddn419","volume":"18","author":"K Tokui","year":"2009","unstructured":"Tokui K, Adachi H, Waza M, Katsuno M, Minamiyama M, Doi H et al (2009) 17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse. Hum Mol Genet 18(5):898\u2013910","journal-title":"Hum Mol Genet"},{"key":"10_CR144","doi-asserted-by":"publisher","first-page":"27703","DOI":"10.1038\/srep27703","volume":"6","author":"LC Bott","year":"2016","unstructured":"Bott LC, Salomons FA, Maric D, Liu Y, Merry D, Fischbeck KH et al (2016) The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC\/C(Cdh1) ubiquitin ligase complex. Sci Rep 6:27703","journal-title":"Sci Rep"},{"issue":"7312","key":"10_CR145","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1038\/nature09299","volume":"467","author":"BH Lee","year":"2010","unstructured":"Lee BH, Lee MJ, Park S, Oh DC, Elsasser S, Chen PC et al (2010) Enhancement of proteasome activity by a small-molecule inhibitor of USP14. Nature (London) 467(7312):179\u2013184","journal-title":"Nature (London)"},{"issue":"4","key":"10_CR146","doi-asserted-by":"publisher","first-page":"497","DOI":"10.1083\/jcb.200505079","volume":"172","author":"Q Qin","year":"2006","unstructured":"Qin Q, Inatome R, Hotta A, Kojima M, Yamamura H, Hirai H et al (2006) A novel GTPase, CRAG, mediates promyelocytic leukemia protein-associated nuclear body formation and degradation of expanded polyglutamine protein. J Cell Biol 172(4):497\u2013504","journal-title":"J Cell Biol"},{"issue":"39","key":"10_CR147","doi-asserted-by":"publisher","first-page":"33879","DOI":"10.1074\/jbc.M111.234997","volume":"286","author":"S Nagashima","year":"2011","unstructured":"Nagashima S, Fukuda T, Kubota Y, Sugiura A, Nakao M, Inatome R et al (2011) CRMP5-associated GTPase (CRAG) protein protects neuronal cells against cytotoxicity of expanded polyglutamine protein partially via c-Fos-dependent activator protein-1 activation. J Biol Chem 286(39):33879\u201333889","journal-title":"J Biol Chem"},{"issue":"45","key":"10_CR148","doi-asserted-by":"publisher","first-page":"10266","DOI":"10.1523\/JNEUROSCI.2734-04.2004","volume":"24","author":"D Goti","year":"2004","unstructured":"Goti D, Katzen SM, Mez J, Kurtis N, Kiluk J, Ben-Haiem L et al (2004) A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration. J Neurosci 24(45):10266\u201310279","journal-title":"J Neurosci"},{"issue":"4","key":"10_CR149","doi-asserted-by":"publisher","first-page":"908","DOI":"10.1111\/j.1471-4159.2004.02369.x","volume":"89","author":"SJ Berke","year":"2004","unstructured":"Berke SJ, Schmied FA, Brunt ER, Ellerby LM, Paulson HL (2004) Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3. J Neurochem 89(4):908\u2013918","journal-title":"J Neurochem"},{"issue":"3","key":"10_CR150","doi-asserted-by":"publisher","first-page":"508","DOI":"10.1093\/hmg\/dds449","volume":"22","author":"J Hubener","year":"2013","unstructured":"Hubener J, Weber JJ, Richter C, Honold L, Weiss A, Murad F et al (2013) Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3). Hum Mol Genet 22(3):508\u2013518","journal-title":"Hum Mol Genet"},{"key":"10_CR151","doi-asserted-by":"publisher","first-page":"543","DOI":"10.1038\/nature10671","volume":"480","author":"P Koch","year":"2011","unstructured":"Koch P, Breuer P, Peitz M, Jungverdorben J, Kesavan J, Poppe D et al (2011) Excitation-induced ataxin-3 aggregation in neurons from patients with Machado\u2013Joseph disease. Nature (London) 480:543","journal-title":"Nature (London)"},{"issue":"26","key":"10_CR152","doi-asserted-by":"publisher","first-page":"18851","DOI":"10.1074\/jbc.M611914200","volume":"282","author":"A Haacke","year":"2007","unstructured":"Haacke A, Hartl FU, Breuer P (2007) Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3. J Biol Chem 282(26):18851\u201318856","journal-title":"J Biol Chem"},{"issue":"8","key":"10_CR153","doi-asserted-by":"publisher","first-page":"2428","DOI":"10.1093\/brain\/aws177","volume":"135","author":"AT Sim\u00f5es","year":"2012","unstructured":"Sim\u00f5es AT, Pereira de Almeida L, Gon\u00e7alves N, Duarte CB, Koeppen A, D\u00e9glon N et al (2012) Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado\u2013Joseph disease. Brain 135(8):2428\u20132439","journal-title":"Brain"},{"issue":"32","key":"10_CR154","doi-asserted-by":"publisher","first-page":"7782","DOI":"10.1523\/JNEUROSCI.1142-17.2017","volume":"37","author":"M Watchon","year":"2017","unstructured":"Watchon M, Yuan KC, Mackovski N, Svahn AJ, Cole NJ, Goldsbury C et al (2017) Calpain inhibition is protective in Machado\u2013Joseph disease zebrafish due to induction of autophagy. J Neurosci 37(32):7782\u20137794","journal-title":"J Neurosci"},{"issue":"18","key":"10_CR155","doi-asserted-by":"publisher","first-page":"4932","DOI":"10.1093\/hmg\/ddu209","volume":"23","author":"AT Sim\u00f5es","year":"2014","unstructured":"Sim\u00f5es AT, Gon\u00e7alves N, Nobre RJ, Duarte CB, Pereira de Almeida L (2014) Calpain inhibition reduces ataxin-3 cleavage alleviating neuropathology and motor impairments in mouse models of Machado\u2013Joseph disease. Hum Mol Genet 23(18):4932\u20134944","journal-title":"Hum Mol Genet"},{"issue":"48","key":"10_CR156","doi-asserted-by":"publisher","first-page":"12713","DOI":"10.1523\/JNEUROSCI.3909-08.2008","volume":"28","author":"X Chen","year":"2008","unstructured":"Chen X, Tang T-S, Tu H, Nelson O, Pook M, Hammer R et al (2008) Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3. J Neurosci 28(48):12713\u201312724","journal-title":"J Neurosci"},{"issue":"11","key":"10_CR157","doi-asserted-by":"publisher","first-page":"E2624","DOI":"10.1073\/pnas.1716071115","volume":"115","author":"AS Sowa","year":"2018","unstructured":"Sowa AS, Martin E, Martins IM, Schmidt J, Depping R, Weber JJ et al (2018) Karyopherin \u03b1-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3. Proc Natl Acad Sci U S A 115(11):E2624\u2013E2E33","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10_CR158","unstructured":"Singh AN, Oehler J, Torrecilla I, Kilgas S, Li S, Vaz B et al (2019) The p97\u2013Ataxin 3 complex regulates homeostasis of the DNA damage response E3 ubiquitin ligase RNF8. EMBO J 38:e102361"},{"key":"10_CR159","unstructured":"Herzog LK, Kevei E, Marchante R, B\u00f6ttcher C, Bindesb\u00bdll C, H\u00e5kon Lystad A et al (2019) The Machado\u2013Joseph disease deubiquitylase ataxin\u20133 interacts with LC3C\/GABARAP and promotes autophagy. Aging Cell 19(1):e13051"}],"container-title":["Advances in Experimental Medicine and Biology","Proteostasis and Disease"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/978-3-030-38266-7_10","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,4,9]],"date-time":"2020-04-09T09:17:19Z","timestamp":1586423839000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/978-3-030-38266-7_10"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020]]},"ISBN":["9783030382650","9783030382667"],"references-count":159,"URL":"https:\/\/doi.org\/10.1007\/978-3-030-38266-7_10","relation":{},"ISSN":["0065-2598","2214-8019"],"issn-type":[{"value":"0065-2598","type":"print"},{"value":"2214-8019","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020]]},"assertion":[{"value":"10 April 2020","order":1,"name":"first_online","label":"First Online","group":{"name":"ChapterHistory","label":"Chapter History"}}]}}