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For: Goti D, Katzen SM, Mez J, Kurtis N, Kiluk J, Ben-Haïem L, Jenkins NA, Copeland NG, Kakizuka A, Sharp AH, Ross CA, Mouton PR, Colomer V. 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 2004;24:10266-79. [PMID: 15537899 DOI: 10.1523/JNEUROSCI.2734-04.2004] [Cited by in Crossref: 147] [Cited by in F6Publishing: 63] [Article Influence: 8.6] [Reference Citation Analysis]
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6 Haas E, Incebacak RD, Hentrich T, Huridou C, Schmidt T, Casadei N, Maringer Y, Bahl C, Zimmermann F, Mills JD, Aronica E, Riess O, Schulze-Hentrich JM, Hübener-Schmid J. A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes. Mol Neurobiol 2021. [PMID: 34716557 DOI: 10.1007/s12035-021-02610-8] [Cited by in Crossref: 1] [Article Influence: 1.0] [Reference Citation Analysis]
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11 Goodwin M, Swanson MS. RNA-binding protein misregulation in microsatellite expansion disorders. Adv Exp Med Biol 2014;825:353-88. [PMID: 25201111 DOI: 10.1007/978-1-4939-1221-6_10] [Cited by in Crossref: 22] [Cited by in F6Publishing: 17] [Article Influence: 2.8] [Reference Citation Analysis]
12 Duarte-Silva S, Neves-Carvalho A, Soares-Cunha C, Teixeira-Castro A, Oliveira P, Silva-Fernandes A, Maciel P. Lithium chloride therapy fails to improve motor function in a transgenic mouse model of Machado-Joseph disease. Cerebellum 2014;13:713-27. [PMID: 25112410 DOI: 10.1007/s12311-014-0589-9] [Cited by in Crossref: 18] [Cited by in F6Publishing: 14] [Article Influence: 2.6] [Reference Citation Analysis]
13 Matilla-Dueñas A, Ashizawa T, Brice A, Magri S, McFarland KN, Pandolfo M, Pulst SM, Riess O, Rubinsztein DC, Schmidt J, Schmidt T, Scoles DR, Stevanin G, Taroni F, Underwood BR, Sánchez I. Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias. Cerebellum 2014;13:269-302. [PMID: 24307138 DOI: 10.1007/s12311-013-0539-y] [Cited by in Crossref: 83] [Cited by in F6Publishing: 69] [Article Influence: 10.4] [Reference Citation Analysis]
14 Nóbrega C, Nascimento-Ferreira I, Onofre I, Albuquerque D, Hirai H, Déglon N, de Almeida LP. Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice. PLoS One 2013;8:e52396. [PMID: 23349684 DOI: 10.1371/journal.pone.0052396] [Cited by in Crossref: 81] [Cited by in F6Publishing: 74] [Article Influence: 9.0] [Reference Citation Analysis]
15 Ingram MA, Orr HT, Clark HB. Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias. Brain Res Bull 2012;88:33-42. [PMID: 21810454 DOI: 10.1016/j.brainresbull.2011.07.016] [Cited by in Crossref: 11] [Cited by in F6Publishing: 10] [Article Influence: 1.0] [Reference Citation Analysis]
16 Moreira R, Mendonça LS, Pereira de Almeida L. Extracellular Vesicles Physiological Role and the Particular Case of Disease-Spreading Mechanisms in Polyglutamine Diseases. Int J Mol Sci 2021;22:12288. [PMID: 34830171 DOI: 10.3390/ijms222212288] [Reference Citation Analysis]
17 Nóbrega C, Nascimento-Ferreira I, Onofre I, Albuquerque D, Déglon N, de Almeida LP. RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease. PLoS One 2014;9:e100086. [PMID: 25144231 DOI: 10.1371/journal.pone.0100086] [Cited by in Crossref: 25] [Cited by in F6Publishing: 22] [Article Influence: 3.1] [Reference Citation Analysis]
18 Chen YS, Hong ZX, Lin SZ, Harn HJ. Identifying Therapeutic Targets for Spinocerebellar Ataxia Type 3/Machado-Joseph Disease through Integration of Pathological Biomarkers and Therapeutic Strategies. Int J Mol Sci 2020;21:E3063. [PMID: 32357546 DOI: 10.3390/ijms21093063] [Cited by in Crossref: 10] [Cited by in F6Publishing: 9] [Article Influence: 5.0] [Reference Citation Analysis]
19 Li YX, Sibon OCM, Dijkers PF. Inhibition of NF-κB in astrocytes is sufficient to delay neurodegeneration induced by proteotoxicity in neurons. J Neuroinflammation 2018;15:261. [PMID: 30205834 DOI: 10.1186/s12974-018-1278-2] [Cited by in Crossref: 18] [Cited by in F6Publishing: 13] [Article Influence: 4.5] [Reference Citation Analysis]
20 Torashima T, Koyama C, Iizuka A, Mitsumura K, Takayama K, Yanagi S, Oue M, Yamaguchi H, Hirai H. Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia. EMBO Rep 2008;9:393-9. [PMID: 18344973 DOI: 10.1038/embor.2008.31] [Cited by in Crossref: 83] [Cited by in F6Publishing: 80] [Article Influence: 5.9] [Reference Citation Analysis]
21 Donaldson J, Powell S, Rickards N, Holmans P, Jones L. What is the Pathogenic CAG Expansion Length in Huntington's Disease? J Huntingtons Dis 2021;10:175-202. [PMID: 33579866 DOI: 10.3233/JHD-200445] [Cited by in Crossref: 2] [Cited by in F6Publishing: 5] [Article Influence: 2.0] [Reference Citation Analysis]
22 Li LB, Yu Z, Teng X, Bonini NM. RNA toxicity is a component of ataxin-3 degeneration in Drosophila. Nature. 2008;453:1107-1111. [PMID: 18449188 DOI: 10.1038/nature06909] [Cited by in Crossref: 223] [Cited by in F6Publishing: 209] [Article Influence: 15.9] [Reference Citation Analysis]
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24 Toonen LJ, Schmidt I, Luijsterburg MS, van Attikum H, van Roon-Mom WM. Antisense oligonucleotide-mediated exon skipping as a strategy to reduce proteolytic cleavage of ataxin-3. Sci Rep 2016;6:35200. [PMID: 27731380 DOI: 10.1038/srep35200] [Cited by in Crossref: 25] [Cited by in F6Publishing: 23] [Article Influence: 4.2] [Reference Citation Analysis]
25 Bento-Torres J, Sobral LL, Reis RR, de Oliveira RB, Anthony DC, Vasconcelos PF, Picanço Diniz CW. Age and Environment Influences on Mouse Prion Disease Progression: Behavioral Changes and Morphometry and Stereology of Hippocampal Astrocytes. Oxid Med Cell Longev 2017;2017:4504925. [PMID: 28243355 DOI: 10.1155/2017/4504925] [Cited by in Crossref: 3] [Cited by in F6Publishing: 4] [Article Influence: 0.6] [Reference Citation Analysis]
26 Kuiper EF, de Mattos EP, Jardim LB, Kampinga HH, Bergink S. Chaperones in Polyglutamine Aggregation: Beyond the Q-Stretch. Front Neurosci 2017;11:145. [PMID: 28386214 DOI: 10.3389/fnins.2017.00145] [Cited by in Crossref: 25] [Cited by in F6Publishing: 23] [Article Influence: 5.0] [Reference Citation Analysis]
27 Guo J, Jiang Z, Biswal BB, Zhou B, Xie D, Gao Q, Sheng W, Chen H, Zhang Y, Fan Y, Wang J, Liu C, Chen H. Hypothalamic Atrophy, Expanded CAG Repeat, and Low Body Mass Index in Spinocerebellar Ataxia Type 3. Movement Disorders. [DOI: 10.1002/mds.29029] [Reference Citation Analysis]
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29 Blum ES, Schwendeman AR, Shaham S. PolyQ disease: misfiring of a developmental cell death program? Trends Cell Biol 2013;23:168-74. [PMID: 23228508 DOI: 10.1016/j.tcb.2012.11.003] [Cited by in Crossref: 40] [Cited by in F6Publishing: 37] [Article Influence: 4.0] [Reference Citation Analysis]
30 Lessing D, Bonini NM. Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila. PLoS Biol 2008;6:e29. [PMID: 18271626 DOI: 10.1371/journal.pbio.0060029] [Cited by in Crossref: 68] [Cited by in F6Publishing: 65] [Article Influence: 4.9] [Reference Citation Analysis]
31 Ramani B, Harris GM, Huang R, Seki T, Murphy GG, Costa Mdo C, Fischer S, Saunders TL, Xia G, McEachin RC, Paulson HL. A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript. Hum Mol Genet 2015;24:1211-24. [PMID: 25320121 DOI: 10.1093/hmg/ddu532] [Cited by in Crossref: 26] [Cited by in F6Publishing: 23] [Article Influence: 3.3] [Reference Citation Analysis]
32 Dansithong W, Paul S, Figueroa KP, Rinehart MD, Wiest S, Pflieger LT, Scoles DR, Pulst SM. Ataxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse model. PLoS Genet 2015;11:e1005182. [PMID: 25902068 DOI: 10.1371/journal.pgen.1005182] [Cited by in Crossref: 52] [Cited by in F6Publishing: 41] [Article Influence: 7.4] [Reference Citation Analysis]
33 Paulson H. Machado-Joseph disease/spinocerebellar ataxia type 3. Handb Clin Neurol 2012;103:437-49. [PMID: 21827905 DOI: 10.1016/B978-0-444-51892-7.00027-9] [Cited by in Crossref: 76] [Cited by in F6Publishing: 44] [Article Influence: 6.9] [Reference Citation Analysis]
34 Boeddrich A, Gaumer S, Haacke A, Tzvetkov N, Albrecht M, Evert BO, Müller EC, Lurz R, Breuer P, Schugardt N, Plassmann S, Xu K, Warrick JM, Suopanki J, Wüllner U, Frank R, Hartl UF, Bonini NM, Wanker EE. An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesis. EMBO J 2006;25:1547-58. [PMID: 16525503 DOI: 10.1038/sj.emboj.7601043] [Cited by in Crossref: 117] [Cited by in F6Publishing: 114] [Article Influence: 7.3] [Reference Citation Analysis]
35 Colomer Gould VF. Mouse models of spinocerebellar ataxia type 3 (Machado-Joseph disease). Neurotherapeutics 2012;9:285-96. [PMID: 22451301 DOI: 10.1007/s13311-012-0117-x] [Cited by in Crossref: 22] [Cited by in F6Publishing: 21] [Article Influence: 2.4] [Reference Citation Analysis]
36 Watchon M, Yuan KC, Mackovski N, Svahn AJ, Cole NJ, Goldsbury C, Rinkwitz S, Becker TS, Nicholson GA, Laird AS. Calpain Inhibition Is Protective in Machado-Joseph Disease Zebrafish Due to Induction of Autophagy. J Neurosci 2017;37:7782-94. [PMID: 28687604 DOI: 10.1523/JNEUROSCI.1142-17.2017] [Cited by in Crossref: 37] [Cited by in F6Publishing: 23] [Article Influence: 7.4] [Reference Citation Analysis]
37 Yang H, Li JJ, Liu S, Zhao J, Jiang YJ, Song AX, Hu HY. Aggregation of polyglutamine-expanded ataxin-3 sequesters its specific interacting partners into inclusions: implication in a loss-of-function pathology. Sci Rep 2014;4:6410. [PMID: 25231079 DOI: 10.1038/srep06410] [Cited by in Crossref: 25] [Cited by in F6Publishing: 25] [Article Influence: 3.1] [Reference Citation Analysis]
38 Colomer Gould VF. Mouse models of Machado-Joseph disease and other polyglutamine spinocerebellar ataxias. NeuroRx 2005;2:480-3. [PMID: 16389311 DOI: 10.1602/neurorx.2.3.480] [Cited by in Crossref: 4] [Cited by in F6Publishing: 2] [Article Influence: 0.3] [Reference Citation Analysis]
39 Jung J, Xu K, Lessing D, Bonini NM. Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3. Hum Mol Genet 2009;18:4843-52. [PMID: 19783548 DOI: 10.1093/hmg/ddp456] [Cited by in Crossref: 46] [Cited by in F6Publishing: 41] [Article Influence: 3.5] [Reference Citation Analysis]
40 Jansen-west K, Todd TW, Daughrity LM, Yue M, Tong J, Carlomagno Y, Del Rosso G, Kurti A, Jones CY, Dunmore JA, Castanedes-casey M, Dickson DW, Wszolek ZK, Fryer JD, Petrucelli L, Prudencio M. Plasma PolyQ-ATXN3 Levels Associate With Cerebellar Degeneration and Behavioral Abnormalities in a New AAV-Based SCA3 Mouse Model. Front Cell Dev Biol 2022;10:863089. [DOI: 10.3389/fcell.2022.863089] [Reference Citation Analysis]
41 Sinnige T, Meisl G, Michaels TCT, Vendruscolo M, Knowles TPJ, Morimoto RI. Kinetic analysis reveals that independent nucleation events determine the progression of polyglutamine aggregation in C. elegans. Proc Natl Acad Sci U S A 2021;118:e2021888118. [PMID: 33836595 DOI: 10.1073/pnas.2021888118] [Cited by in Crossref: 1] [Article Influence: 1.0] [Reference Citation Analysis]
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43 Bettencourt C, Santos C, Montiel R, Costa Mdo C, Cruz-Morales P, Santos LR, Simões N, Kay T, Vasconcelos J, Maciel P, Lima M. Increased transcript diversity: novel splicing variants of Machado-Joseph disease gene (ATXN3). Neurogenetics 2010;11:193-202. [PMID: 19714377 DOI: 10.1007/s10048-009-0216-y] [Cited by in Crossref: 22] [Cited by in F6Publishing: 20] [Article Influence: 1.7] [Reference Citation Analysis]
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47 Gillis J, Schipper-Krom S, Juenemann K, Gruber A, Coolen S, van den Nieuwendijk R, van Veen H, Overkleeft H, Goedhart J, Kampinga HH, Reits EA. The DNAJB6 and DNAJB8 protein chaperones prevent intracellular aggregation of polyglutamine peptides. J Biol Chem 2013;288:17225-37. [PMID: 23612975 DOI: 10.1074/jbc.M112.421685] [Cited by in Crossref: 77] [Cited by in F6Publishing: 46] [Article Influence: 8.6] [Reference Citation Analysis]
48 Bettencourt C, Raposo M, Kazachkova N, Santos C, Kay T, Vasconcelos J, Maciel P, Donis KC, Saraiva-Pereira ML, Jardim LB, Sequeiros J, Bruges-Armas J, Lima M. Sequence analysis of 5' regulatory regions of the Machado-Joseph disease gene (ATXN3). Cerebellum 2012;11:1045-50. [PMID: 22422287 DOI: 10.1007/s12311-012-0373-7] [Cited by in Crossref: 6] [Cited by in F6Publishing: 6] [Article Influence: 0.7] [Reference Citation Analysis]
49 Silva-Fernandes A, Duarte-Silva S, Neves-Carvalho A, Amorim M, Soares-Cunha C, Oliveira P, Thirstrup K, Teixeira-Castro A, Maciel P. Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease. Neurotherapeutics 2014;11:433-49. [PMID: 24477711 DOI: 10.1007/s13311-013-0255-9] [Cited by in Crossref: 65] [Cited by in F6Publishing: 63] [Article Influence: 8.1] [Reference Citation Analysis]
50 Havel LS, Li S, Li XJ. Nuclear accumulation of polyglutamine disease proteins and neuropathology. Mol Brain 2009;2:21. [PMID: 19575804 DOI: 10.1186/1756-6606-2-21] [Cited by in Crossref: 28] [Cited by in F6Publishing: 25] [Article Influence: 2.2] [Reference Citation Analysis]
51 Christie NT, Lee AL, Fay HG, Gray AA, Kikis EA. Novel polyglutamine model uncouples proteotoxicity from aging. PLoS One 2014;9:e96835. [PMID: 24817148 DOI: 10.1371/journal.pone.0096835] [Cited by in Crossref: 16] [Cited by in F6Publishing: 14] [Article Influence: 2.0] [Reference Citation Analysis]
52 Martins AC, Rieck M, Leotti VB, Saraiva-Pereira ML, Jardim LB. Variants in Genes of Calpain System as Modifiers of Spinocerebellar Ataxia Type 3 Phenotype. J Mol Neurosci 2021;71:1906-13. [PMID: 34191270 DOI: 10.1007/s12031-021-01877-9] [Reference Citation Analysis]
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55 Harris GM, Dodelzon K, Gong L, Gonzalez-Alegre P, Paulson HL. Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. PLoS One 2010;5:e13695. [PMID: 21060878 DOI: 10.1371/journal.pone.0013695] [Cited by in Crossref: 44] [Cited by in F6Publishing: 42] [Article Influence: 3.7] [Reference Citation Analysis]
56 Carmona V, Cunha-Santos J, Onofre I, Simões AT, Vijayakumar U, Davidson BL, Pereira de Almeida L. Unravelling Endogenous MicroRNA System Dysfunction as a New Pathophysiological Mechanism in Machado-Joseph Disease. Mol Ther 2017;25:1038-55. [PMID: 28236575 DOI: 10.1016/j.ymthe.2017.01.021] [Cited by in Crossref: 26] [Cited by in F6Publishing: 21] [Article Influence: 5.2] [Reference Citation Analysis]
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