{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T12:04:39Z","timestamp":1779365079818,"version":"3.53.0"},"publisher-location":"New York, NY","reference-count":114,"publisher":"Springer New York","isbn-type":[{"value":"9781493978243","type":"print"},{"value":"9781493978250","type":"electronic"}],"license":[{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"unspecified","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":[[2018]]},"DOI":"10.1007\/978-1-4939-7825-0_5","type":"book-chapter","created":{"date-parts":[[2018,5,31]],"date-time":"2018-05-31T12:44:16Z","timestamp":1527770656000},"page":"75-96","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Nonmammalian Models of Huntington\u2019s Disease"],"prefix":"10.1007","author":[{"given":"Anjalika","family":"Chongtham","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brett","family":"Barbaro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomas","family":"Filip","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adeela","family":"Syed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weijian","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne R.","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Lawrence","family":"Marsh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2018,6,1]]},"reference":[{"key":"5_CR1","doi-asserted-by":"crossref","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"},{"key":"5_CR2","doi-asserted-by":"crossref","first-page":"3401","DOI":"10.4161\/cc.9.17.12718","volume":"9","author":"JS Steffan","year":"2010","unstructured":"Steffan JS (2010) Does Huntingtin play a role in selective macroautophagy? Cell Cycle 9:3401\u20133413","journal-title":"Cell Cycle"},{"key":"5_CR3","doi-asserted-by":"crossref","first-page":"16889","DOI":"10.1073\/pnas.1420103111","volume":"111","author":"J Ochaba","year":"2014","unstructured":"Ochaba J, Lukacsovich T, Csiko G et al (2014) Potential function for the Huntingtin protein as a scaffold for selective autophagy. Proc Natl Acad Sci U S A 111:16889\u201316894","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR4","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1038\/ncb3101","volume":"17","author":"YN Rui","year":"2015","unstructured":"Rui YN, Xu Z, Patel B et al (2015) Huntingtin functions as a scaffold for selective macroautophagy. Nat Cell Biol 17:262\u2013275","journal-title":"Nat Cell Biol"},{"key":"5_CR5","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","volume":"72","author":"The Huntington\u2019s Disease Collaborative Research Group","year":"1993","unstructured":"The Huntington\u2019s Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes. Cell 72:971\u2013983","journal-title":"Cell"},{"key":"5_CR6","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1002\/bies.20029","volume":"26","author":"JL Marsh","year":"2004","unstructured":"Marsh JL, Thompson LM (2004) Can flies help humans treat neurodegenerative diseases? Bioessays 26:485\u2013496","journal-title":"Bioessays"},{"key":"5_CR7","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","volume":"87","author":"L Mangiarini","year":"1996","unstructured":"Mangiarini L, Sathasivam K, Selle M et al (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87:493\u2013506","journal-title":"Cell"},{"key":"5_CR8","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1002\/1531-8249(200001)47:1<80::AID-ANA13>3.0.CO;2-K","volume":"47","author":"SJ Tabrizi","year":"2000","unstructured":"Tabrizi SJ, Workman J, Har PE et al (2000) Mitochondrial dysfunction and free radical damage in the Huntington R6\/2 transgenic mouse. Ann Neurol 47:80\u201386","journal-title":"Ann Neurol"},{"key":"5_CR9","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1093\/hmg\/10.2.137","volume":"10","author":"CH Lin","year":"2001","unstructured":"Lin CH, Tallaksen-Greene S, Chien WM et al (2001) Neurological abnormalities in a knock-in mouse model of Huntington\u2019s disease. Hum Mol Genet 10:137\u2013144","journal-title":"Hum Mol Genet"},{"key":"5_CR10","doi-asserted-by":"crossref","first-page":"3063","DOI":"10.1093\/hmg\/ddl248","volume":"15","author":"A Solans","year":"2006","unstructured":"Solans A, Zambrano A, Rodrigue M, Barrientos A (2006) Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III. Hum Mol Genet 15:3063\u20133081","journal-title":"Hum Mol Genet"},{"key":"5_CR11","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1093\/hmg\/ddn404","volume":"18","author":"H Wang","year":"2009","unstructured":"Wang H, Lim PJ, Karbowski M, Monteiro MJ (2009) Effects of overexpression of huntingtin proteins on mitochondrial integrity. Hum Mol Genet 18:737\u2013752","journal-title":"Hum Mol Genet"},{"key":"5_CR12","doi-asserted-by":"crossref","first-page":"3919","DOI":"10.1093\/hmg\/ddq306","volume":"19","author":"J Kim","year":"2010","unstructured":"Kim J, Moody JP, Edgerly CK et al (2010) Mitochondrial loss, dysfunction and altered dynamics in Huntington\u2019s disease. Hum Mol Genet 19:3919\u20133935","journal-title":"Hum Mol Genet"},{"key":"5_CR13","doi-asserted-by":"crossref","first-page":"3372","DOI":"10.1093\/hmg\/ddq249","volume":"19","author":"MT Besson","year":"2010","unstructured":"Besson MT, Dupont P, Fridell YW, Lievens JC (2010) Increased energy metabolism rescues glia-induced pathology in a Drosophila model of Huntington\u2019s disease. Hum Mol Genet 19:3372\u20133382","journal-title":"Hum Mol Genet"},{"key":"5_CR14","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/S0896-6273(03)00594-4","volume":"40","author":"S Gunawardena","year":"2003","unstructured":"Gunawardena S, Her LS, Brusch RG et al (2003) Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila. Neuron 40:25\u201340","journal-title":"Neuron"},{"key":"5_CR15","doi-asserted-by":"crossref","DOI":"10.1038\/cddis.2014.581","volume":"6","author":"B Khalil","year":"2015","unstructured":"Khalil B, El Fissi N, Aouane A et al (2015) PINK1-induced mitophagy promotes neuroprotection in Huntington\u2019s disease. Cell Death Dis 6:e1617","journal-title":"Cell Death Dis"},{"key":"5_CR16","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/35040584","volume":"408","author":"P Fernandez-Funez","year":"2000","unstructured":"Fernandez-Funez P, Nino-Rosales ML, de Gouyon B et al (2000) Identification of genes that modify ataxin-1-induced neurodegeneration. Nature 408:101\u2013106","journal-title":"Nature"},{"key":"5_CR17","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1038\/35099568","volume":"413","author":"JS Steffan","year":"2001","unstructured":"Steffan JS, Bodai L, Pallos J et\u00a0al (2001) Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 413:739\u2013743","journal-title":"Nature"},{"key":"5_CR18","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1016\/j.tibs.2013.05.003","volume":"38","author":"J Labbadia","year":"2013","unstructured":"Labbadia J, Morimoto RI (2013) Huntington\u2019s disease: underlying molecular mechanisms and emerging concepts. Trends Biochem Sci 38:378\u2013385","journal-title":"Trends Biochem Sci"},{"key":"5_CR19","doi-asserted-by":"crossref","first-page":"2830","DOI":"10.1523\/JNEUROSCI.3344-05.2006","volume":"26","author":"EA Bates","year":"2006","unstructured":"Bates EA, Victor M, Jones AK et al (2006) Differential contributions of Caenorhabditis elegans 35 histone deacetylases to huntingtin polyglutamine toxicity. J Neurosci 26:2830\u20132838","journal-title":"J Neurosci"},{"key":"5_CR20","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1038\/nature01960","volume":"425","author":"KT Howitz","year":"2003","unstructured":"Howitz KT, Bitterman KJ, Cohen HY et al (2003) Small molecule activators of sirtuins extend Saccharomyces cerevisiae lifespan. Nature 425:191\u2013196","journal-title":"Nature"},{"key":"5_CR21","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1038\/ng1534","volume":"37","author":"JA Parker","year":"2005","unstructured":"Parker JA, Arango M, Abderrahmane S et al (2005) Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons. Nat Genet 37:349\u2013350","journal-title":"Nat Genet"},{"key":"5_CR22","doi-asserted-by":"crossref","first-page":"3767","DOI":"10.1093\/hmg\/ddn273","volume":"17","author":"J Pallos","year":"2008","unstructured":"Pallos J, Bodai L, Lukacsovich T et al (2008) Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington\u2019s disease. Hum Mol Genet 17:3767\u20133775","journal-title":"Hum Mol Genet"},{"key":"5_CR23","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1038\/464480a","volume":"464","author":"H Ledford","year":"2010","unstructured":"Ledford H (2010) Ageing: much ado about ageing. Nature 464:480\u2013481","journal-title":"Nature"},{"key":"5_CR24","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1086\/378133","volume":"73","author":"JL Li","year":"2003","unstructured":"Li JL, Hayden MR, Almqvist EW et al (2003) A genome scan for modifiers of age at onset in Huntington disease: the HD MAPS study. Am J Hum Genet 73:682\u2013687","journal-title":"Am J Hum Genet"},{"key":"5_CR25","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/j.ajhg.2015.12.018","volume":"98","author":"JW Keum","year":"2016","unstructured":"Keum JW, Shin A, Gillis T et al (2016) The HTT CAG-expansion mutation determines age at death but not disease duration in Huntington disease. Am J Hum Genet 98:287\u2013298","journal-title":"Am J Hum Genet"},{"key":"5_CR26","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1002\/brb3.235","volume":"4","author":"EA Skillings","year":"2014","unstructured":"Skillings EA, Wood NI, Morton AJ (2014) Beneficial effects of environmental enrichment and food entrainment in the R6\/2 mouse model of Huntington\u2019s disease. Brain Behav 4:675\u2013686","journal-title":"Brain Behav"},{"key":"5_CR27","doi-asserted-by":"crossref","first-page":"2270","DOI":"10.1523\/JNEUROSCI.1658-03.2004","volume":"24","author":"TL Spires","year":"2004","unstructured":"Spires TL, Grote HE, Varshney NK, Cordery PM et al (2004) Environmental enrichment rescues protein deficits in a mouse model of Huntington\u2019s disease, indicating a possible disease mechanism. J Neurosci 24:2270\u20132276","journal-title":"J Neurosci"},{"key":"5_CR28","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1093\/hmg\/ddm315","volume":"17","author":"J Branco","year":"2008","unstructured":"Branco J, Al-Ramahi I, Ukani L et al (2008) Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum Mol Genet 17:376\u2013390","journal-title":"Hum Mol Genet"},{"key":"5_CR29","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.0030082","volume":"3","author":"LS Kaltenbach","year":"2007","unstructured":"Kaltenbach LS, Romero E, Becklin RR et al (2007) Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet 3:e82","journal-title":"PLoS Genet"},{"key":"5_CR30","doi-asserted-by":"crossref","first-page":"2995","DOI":"10.1093\/hmg\/ddu010","volume":"23","author":"MR Smith","year":"2014","unstructured":"Smith MR, Syed A, Lukacsovich T et al (2014) A potent and selective Sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington\u2019s disease. Hum Mol Genet 23:2995\u20133007","journal-title":"Hum Mol Genet"},{"key":"5_CR31","doi-asserted-by":"crossref","first-page":"2041","DOI":"10.1073\/pnas.0437870100","volume":"100","author":"E Hockly","year":"2003","unstructured":"Hockly E, Richon VM, Woodman B et al (2003) Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington\u2019s disease. Proc Natl Acad Sci U S A 100:2041\u20132046","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR32","doi-asserted-by":"crossref","first-page":"194","DOI":"10.3109\/01677063.2010.514367","volume":"24","author":"Y Nishimura","year":"2010","unstructured":"Nishimura Y, Yalgin C, Akimoto S et al (2010) Selection of behaviors and segmental coordination during larval locomotion is disrupted by nuclear polyglutamine inclusions in a new Drosophila Huntington\u2019s disease-like model. J Neurogenet 24:194\u2013206","journal-title":"J Neurogenet"},{"key":"5_CR33","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1016\/j.neuro.2008.04.006","volume":"29","author":"RT Peterson","year":"2008","unstructured":"Peterson RT, Nass R, Boyd WA et al (2008) Use of non-mammalian alternative models for neurotoxicological study. Neurotoxicology 29:546\u2013555","journal-title":"Neurotoxicology"},{"key":"5_CR34","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1038\/nrn3570","volume":"14","author":"MA Pouladi","year":"2013","unstructured":"Pouladi MA, Morton AJ, Hayden MR (2013) Choosing an animal model for the study of Huntington\u2019s disease. Nat Rev Neurosci 14:708\u2013721","journal-title":"Nat Rev Neurosci"},{"key":"5_CR35","doi-asserted-by":"crossref","first-page":"26136","DOI":"10.1074\/jbc.M112.362764","volume":"287","author":"M Beam","year":"2012","unstructured":"Beam M, Silva MC, Morimoto RI (2012) Dynamic imaging by fluorescence correlation spectroscopy identifies diverse populations of polyglutamine oligomers formed in vivo. J Biol Chem 287:26136\u201326145","journal-title":"J Biol Chem"},{"key":"5_CR36","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1007\/978-0-387-39975-1_15","volume":"594","author":"HR Brignull","year":"2007","unstructured":"Brignull HR, Morley JF, Morimoto RI (2007) The stress of misfolded proteins: C. elegans models for neurodegenerative disease and aging. Adv Exp Med Biol 594:167\u2013189","journal-title":"Adv Exp Med Biol"},{"key":"5_CR37","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1186\/1741-7007-11-100","volume":"11","author":"T Gidalevitz","year":"2013","unstructured":"Gidalevitz T, Wang N, Deravaj T et al (2013) Natural genetic variation determines susceptibility to aggregation or toxicity in a C. elegans model for polyglutamine disease. BMC Biol 11:100","journal-title":"BMC Biol"},{"key":"5_CR38","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1093\/hmg\/ddu504","volume":"24","author":"BA Barbaro","year":"2015","unstructured":"Barbaro BA, Lukacsovich T, Agrawal N et al (2015) Comparative study of naturally occurring huntingtin fragments in Drosophila points to exon 1 as the most pathogenic species in Huntington\u2019s disease. Hum Mol Genet 24:913\u2013925","journal-title":"Hum Mol Genet"},{"key":"5_CR39","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1038\/nrn2809","volume":"11","author":"V Khurana","year":"2010","unstructured":"Khurana V, Lindquist S (2010) Modelling neurodegeneration in Saccharomyces cerevisiae: why cook with baker\u2019s yeast? Nat Rev Neurosci 11:436\u2013449","journal-title":"Nat Rev Neurosci"},{"key":"5_CR40","doi-asserted-by":"crossref","first-page":"269","DOI":"10.4161\/pri.18005","volume":"5","author":"RP Mason","year":"2011","unstructured":"Mason RP, Giorgini F (2011) Modeling Huntington disease in yeast: perspectives and future directions. Prion 5:269\u2013276","journal-title":"Prion"},{"key":"5_CR41","doi-asserted-by":"crossref","first-page":"941232","DOI":"10.1155\/2012\/941232","volume":"2012","author":"C Pereira","year":"2012","unstructured":"Pereira C, Bessa C, Soare J et\u00a0al (2012) Contribution of yeast models to neurodegeneration research. J Biomed Biotechnol 2012:941232","journal-title":"J Biomed Biotechnol"},{"key":"5_CR42","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1002\/biot.200700217","volume":"3","author":"L Miller-Fleming","year":"2008","unstructured":"Miller-Fleming L, Giorgini F, Outeiro TF (2008) Yeast as a model for studying human neurodegenerative disorders. Biotechnol J 3:325\u2013338","journal-title":"Biotechnol J"},{"key":"5_CR43","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1007\/s10522-016-9666-4","volume":"18","author":"AV Oliveira","year":"2017","unstructured":"Oliveira AV, Vilaca R, Santos CN et al (2017) Exploring the power of yeast to model aging and age-related neurodegenerative disorders. Biogerontology 18:3\u201334","journal-title":"Biogerontology"},{"key":"5_CR44","doi-asserted-by":"crossref","first-page":"3308","DOI":"10.1101\/gad.1673408","volume":"22","author":"ML Duennwald","year":"2008","unstructured":"Duennwald ML, Lindquist S (2008) Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity. Genes Dev 22:3308\u20133319","journal-title":"Genes Dev"},{"key":"5_CR45","doi-asserted-by":"crossref","first-page":"7554","DOI":"10.1128\/MCB.23.21.7554-7565.2003","volume":"23","author":"AB Meriin","year":"2003","unstructured":"Meriin AB, Zhang X, Miliaras NB et al (2003) Aggregation of expanded polyglutamine domain in yeast leads to defects in endocytosis. Mol Cell Biol 23:7554\u20137565","journal-title":"Mol Cell Biol"},{"key":"5_CR46","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1016\/j.bbabio.2006.05.004","volume":"1757","author":"S Sokolov","year":"2006","unstructured":"Sokolov S, Pozniakovsky A, Bocharova N et al (2006) Expression of an expanded polyglutamine domain in yeast causes death with apoptotic markers. Biochim Biophys Acta 1757:660\u2013666","journal-title":"Biochim Biophys Acta"},{"key":"5_CR47","doi-asserted-by":"crossref","first-page":"16685","DOI":"10.1073\/pnas.0707842104","volume":"104","author":"V Chopra","year":"2007","unstructured":"Chopra V, Fox JH, Lieberman G et al (2007) A small-molecule therapeutic lead for Huntington\u2019s disease: preclinical pharmacology and efficacy of C2-8 in the R6\/2 transgenic mouse. Proc Natl Acad Sci U S A 104:16685\u201316689","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR48","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.1096\/fj.09-148601","volume":"24","author":"A Ocampo","year":"2010","unstructured":"Ocampo A, Zambrano A, Barrientos A (2010) Suppression of polyglutamine-induced cytotoxicity in Saccharomyces cerevisiae by enhancement of mitochondrial biogenesis. FASEB J 24:1431\u20131441","journal-title":"FASEB J"},{"key":"5_CR49","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1016\/j.cell.2011.05.020","volume":"145","author":"D Zwilling","year":"2011","unstructured":"Zwilling D, Huang SY, Sathyasaikumar KV et al (2011) Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration. Cell 145:863\u2013874","journal-title":"Cell"},{"key":"5_CR50","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1038\/ng1542","volume":"37","author":"F Giorgini","year":"2005","unstructured":"Giorgini F, Guidetti P, Nguyen Q et al (2005) A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease. Nat Genet 37:526\u2013531","journal-title":"Nat Genet"},{"key":"5_CR51","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1073\/pnas.0408936102","volume":"102","author":"X Zhang","year":"2005","unstructured":"Zhang X, Smith DL, Meriin AB et al (2005) A potent small molecule inhibits polyglutamine aggregation in Huntington\u2019s disease neurons and suppresses neurodegeneration in vivo. Proc Natl Acad Sci U S A 102:892\u2013897","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR52","doi-asserted-by":"crossref","first-page":"2743","DOI":"10.1093\/hmg\/ddl210","volume":"15","author":"DE Ehrnhoefer","year":"2006","unstructured":"Ehrnhoefer DE, Duennwald M, Markovic P et al (2006) Green tea (\u2212)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington\u2019s disease models. Hum Mol Genet 15:2743\u20132751","journal-title":"Hum Mol Genet"},{"key":"5_CR53","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1038\/nrd2031","volume":"5","author":"T Kaletta","year":"2006","unstructured":"Kaletta T, Hengartner MO (2006) Finding function in novel targets: C. elegans as a model organism. Nat Rev Drug Discov 5:387\u2013398","journal-title":"Nat Rev Drug Discov"},{"key":"5_CR54","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/B978-0-12-384878-9.00015-7","volume":"100","author":"B Wolozin","year":"2011","unstructured":"Wolozin B, Gabel C, Ferree A et al (2011) Watching worms whither: modeling neurodegeneration in C. elegans. Prog Mol Biol Transl Sci 100:499\u2013514","journal-title":"Prog Mol Biol Transl Sci"},{"key":"5_CR55","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1186\/s13065-015-0143-y","volume":"9","author":"X Chen","year":"2015","unstructured":"Chen X, Barclay JW, Burgoyne RD, Morgan A (2015) Using C. elegans to discover therapeutic compounds for ageing-associated neurodegenerative diseases. Chem Cent J 9:65","journal-title":"Chem Cent J"},{"key":"5_CR56","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1093\/genetics\/77.1.71","volume":"77","author":"S Brenner","year":"1974","unstructured":"Brenner S (1974) The genetics of Caenorhabditis elegans. Genetics 77:71\u201394","journal-title":"Genetics"},{"key":"5_CR57","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1186\/1750-1326-4-38","volume":"4","author":"D Teschendorf","year":"2009","unstructured":"Teschendorf D, Link CD (2009) What have worm models told us about the mechanisms of neuronal dysfunction in human neurodegenerative diseases? Mol Neurodegener 4:38","journal-title":"Mol Neurodegener"},{"key":"5_CR58","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/j.nbd.2010.05.012","volume":"40","author":"M Dimitriadi","year":"2010","unstructured":"Dimitriadi M, Hart AC (2010) Neurodegenerative disorders: insights from the nematode Caenorhabditis elegans. Neurobiol Dis 40:4\u201311","journal-title":"Neurobiol Dis"},{"key":"5_CR59","doi-asserted-by":"crossref","first-page":"279","DOI":"10.3389\/fgene.2014.00279","volume":"5","author":"AG Alexander","year":"2014","unstructured":"Alexander AG, Marfil V, Li C (2014) Use of Caenorhabditis elegans as a model to study Alzheimer\u2019s disease and other neurodegenerative diseases. Front Genet 5:279","journal-title":"Front Genet"},{"key":"5_CR60","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1073\/pnas.96.1.179","volume":"96","author":"PW Faber","year":"1999","unstructured":"Faber PW, Alter JR, MacDonald ME, Hart AC (1999) Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Proc Natl Acad Sci U S A 96:179\u2013184","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR61","doi-asserted-by":"crossref","first-page":"13318","DOI":"10.1073\/pnas.231476398","volume":"98","author":"JA Parker","year":"2001","unstructured":"Parker JA, Connolly JB, Wellington C et al (2001) Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Proc Natl Acad Sci U S A 98:13318\u201313323","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR62","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1126\/science.1124514","volume":"311","author":"T Gidalevitz","year":"2006","unstructured":"Gidalevitz T, Ben-Zvi A, Ho KH et al (2006) Progressive disruption of cellular protein folding in models of polyglutamine diseases. Science 311:1471\u20131474","journal-title":"Science"},{"key":"5_CR63","doi-asserted-by":"publisher","unstructured":"Vayndorf EM, Scerbak C, Hunter S et al (2016) Morphological remodeling of C. elegans neurons during aging is modified by compromised protein homeostasis. NPJ Aging Mech Dis 2. https:\/\/doi.org\/10.1038\/npjamd.2016.1","DOI":"10.1038\/npjamd.2016.1"},{"key":"5_CR64","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1093\/hmg\/ddv513","volume":"25","author":"RP Vazquez-Manrique","year":"2016","unstructured":"Vazquez-Manrique RP, Farina F, Cambon K et al (2016) AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington\u2019s disease. Hum Mol Genet 25:1043\u20131058","journal-title":"Hum Mol Genet"},{"key":"5_CR65","doi-asserted-by":"crossref","first-page":"12630","DOI":"10.1523\/JNEUROSCI.0277-12.2012","volume":"32","author":"JA Parker","year":"2012","unstructured":"Parker JA, Vazquez-Manrique RP, Tourette C et al (2012) Integration of beta-catenin, sirtuin, and FOXO signaling protects from mutant huntingtin toxicity. J Neurosci 32:12630\u201312640","journal-title":"J Neurosci"},{"key":"5_CR66","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1186\/1471-2164-13-91","volume":"13","author":"FX Lejeune","year":"2012","unstructured":"Lejeune FX, Mesrob L, Parmentier F et al (2012) Large-scale functional RNAi screen in C. elegans identifies genes that regulate the dysfunction of mutant polyglutamine neurons. BMC Genomics 13:91","journal-title":"BMC Genomics"},{"key":"5_CR67","doi-asserted-by":"crossref","unstructured":"Neri C (2011) Value of invertebrate genetics and biology to develop neuroprotective and preventive medicine in Huntington\u2019s disease. In: Lo DC, Hughes RE (eds) Neurobiology of Huntington\u2019s disease: applications to drug discovery, Boca Raton, FL, CRC Press\/Taylor & Francis","DOI":"10.1201\/EBK0849390005-c6"},{"key":"5_CR68","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1093\/hmg\/ddq464","volume":"20","author":"VM Gohil","year":"2011","unstructured":"Gohil VM, Offner N, Walker JA et al (2011) Meclizine is neuroprotective in models of Huntington\u2019s disease. Hum Mol Genet 20:294\u2013300","journal-title":"Hum Mol Genet"},{"key":"5_CR69","doi-asserted-by":"crossref","first-page":"7927","DOI":"10.1073\/pnas.1002924107","volume":"107","author":"R Luthi-Carter","year":"2010","unstructured":"Luthi-Carter R, Taylor DM, Pallos J et\u00a0al (2010) SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis. Proc Natl Acad Sci U S A 107:7927\u20137932","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR70","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.jneumeth.2015.07.026","volume":"265","author":"EA Lewis","year":"2016","unstructured":"Lewis EA, Smith GA (2016) Using Drosophila models of Huntington\u2019s disease as a translatable tool. J Neurosci Methods 265:89\u201398","journal-title":"J Neurosci Methods"},{"key":"5_CR71","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.expneurol.2015.08.002","volume":"274","author":"Z Xu","year":"2015","unstructured":"Xu Z, Tito AJ, Rui YN, Zhang S (2015) Studying polyglutamine diseases in Drosophila. Exp Neurol 274:25\u201341","journal-title":"Exp Neurol"},{"key":"5_CR72","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1146\/annurev.pathol.3.121806.151529","volume":"4","author":"B Lu","year":"2009","unstructured":"Lu B, Vogel H (2009) Drosophila models of neurodegenerative diseases. Annu Rev Pathol 4:315\u2013342","journal-title":"Annu Rev Pathol"},{"key":"5_CR73","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1038\/sj.cdd.4400757","volume":"7","author":"HY Chan","year":"2000","unstructured":"Chan HY, Bonini NM (2000) Drosophila models of human neurodegenerative disease. Cell Death Differ 7:1075\u20131080","journal-title":"Cell Death Differ"},{"key":"5_CR74","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1126\/science.1092194","volume":"304","author":"JS Steffan","year":"2004","unstructured":"Steffan JS, Agrawal N, Pallos J et\u00a0al (2004) SUMO modification of Huntingtin and Huntington\u2019s disease pathology. Science 304:100\u2013104","journal-title":"Science"},{"key":"5_CR75","doi-asserted-by":"crossref","first-page":"R187","DOI":"10.1093\/hmg\/ddg271","volume":"12 Spec No 2","author":"JL Marsh","year":"2003","unstructured":"Marsh JL, Pallos J, Thompson LM (2003) Fly models of Huntington\u2019s disease. Hum Mol Genet 12 Spec No 2:R187\u2013R193","journal-title":"Hum Mol Genet"},{"key":"5_CR76","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1534\/genetics.115.179457","volume":"201","author":"L McGurk","year":"2015","unstructured":"McGurk L, Berson A, Bonini NM (2015) Drosophila as an in vivo model for human neurodegenerative disease. Genetics 201:377\u2013402","journal-title":"Genetics"},{"key":"5_CR77","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1242\/dev.118.2.401","volume":"118","author":"AH Brand","year":"1993","unstructured":"Brand AH, Perrimon N (1993) Targeted gene expression as a means of altering cell fates and generating dominant phenotypes. Development 118:401\u2013415","journal-title":"Development"},{"key":"5_CR78","doi-asserted-by":"crossref","first-page":"5199","DOI":"10.1073\/pnas.0700895104","volume":"104","author":"W Song","year":"2007","unstructured":"Song W, Onishi M, Jan LY, Jan YN (2007) Peripheral multidendritic sensory neurons are necessary for rhythmic locomotion behavior in Drosophila larvae. Proc Natl Acad Sci U S A 104:5199\u20135204","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR79","doi-asserted-by":"crossref","first-page":"9418","DOI":"10.1523\/JNEUROSCI.23-28-09418.2003","volume":"23","author":"RJ Ferrante","year":"2003","unstructured":"Ferrante RJ, Kubilus JK, Lee J et\u00a0al (2003) Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington\u2019s disease mice. J Neurosci 23:9418\u20139427","journal-title":"J Neurosci"},{"key":"5_CR80","doi-asserted-by":"crossref","first-page":"403","DOI":"10.2174\/156720506778249407","volume":"3","author":"G Sadri-Vakili","year":"2006","unstructured":"Sadri-Vakili G, Cha JH (2006) Histone deacetylase inhibitors: a novel therapeutic approach to Huntington's disease (complex mechanism of neuronal death). Curr Alzheimer Res 3:403\u2013408","journal-title":"Curr Alzheimer Res"},{"key":"5_CR81","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pbio.1001717","volume":"11","author":"M Mielcarek","year":"2013","unstructured":"Mielcarek M, Landles C, Weiss A et al (2013) HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration. PLoS Biol 11:e1001717","journal-title":"PLoS Biol"},{"key":"5_CR82","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0044498","volume":"7","author":"M Beconi","year":"2012","unstructured":"Beconi M, Aziz O, Matthews K et al (2012) Oral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivo. PLoS One 7:e44498","journal-title":"PLoS One"},{"key":"5_CR83","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0031080","volume":"7","author":"L Moumne","year":"2012","unstructured":"Moumne L, Campbell K, Howland D et al (2012) Genetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington\u2019s disease. PLoS One 7:e31080","journal-title":"PLoS One"},{"key":"5_CR84","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0027746","volume":"6","author":"M Mielcarek","year":"2011","unstructured":"Mielcarek M, Benn CL, Franklin SA et al (2011) SAHA decreases HDAC 2 and 4 levels in vivo and improves molecular phenotypes in the R6\/2 mouse model of Huntington\u2019s disease. PLoS One 6:e27746","journal-title":"PLoS One"},{"key":"5_CR85","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0020696","volume":"6","author":"A Bobrowska","year":"2011","unstructured":"Bobrowska A, Paganetti P, Matthias P, Bates GP (2011) Hdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6\/2 mouse model of Huntington\u2019s disease. PLoS One 6:e20696","journal-title":"PLoS One"},{"key":"5_CR86","volume":"4","author":"CL Benn","year":"2009","unstructured":"Benn CL, Butler R, Mariner L et al (2009) Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6\/2 mouse model of Huntington\u2019s disease. PLoS One 4:e5747","journal-title":"PLoS One"},{"key":"5_CR87","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/j.neuron.2010.06.021","volume":"67","author":"JP Miller","year":"2010","unstructured":"Miller JP, Holcomb J, Al-Ramahi I et al (2010) Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington\u2019s disease. Neuron 67:199\u2013212","journal-title":"Neuron"},{"key":"5_CR88","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1038\/nchembio.1790","volume":"11","author":"M Jimenez-Sanchez","year":"2015","unstructured":"Jimenez-Sanchez M, Lam W, Hannus M et al (2015) siRNA screen identifies QPCT as a druggable target for Huntington\u2019s disease. Nat Chem Biol 11:347\u2013354","journal-title":"Nat Chem Biol"},{"key":"5_CR89","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.nbd.2010.05.017","volume":"40","author":"O Bandmann","year":"2010","unstructured":"Bandmann O, Burton EA (2010) Genetic zebrafish models of neurodegenerative diseases. Neurobiol Dis 40:58\u201365","journal-title":"Neurobiol Dis"},{"key":"5_CR90","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1002\/dneu.20888","volume":"72","author":"TS Becker","year":"2012","unstructured":"Becker TS, Rinkwitz S (2012) Zebrafish as a genomics model for human neurological and polygenic disorders. Dev Neurobiol 72:415\u2013428","journal-title":"Dev Neurobiol"},{"key":"5_CR91","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1002\/bies.20615","volume":"29","author":"P Chapouton","year":"2007","unstructured":"Chapouton P, Jagasia R, Bally-Cuif L (2007) Adult neurogenesis in non-mammalian vertebrates. Bioessays 29:745\u2013757","journal-title":"Bioessays"},{"key":"5_CR92","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.bbadis.2010.09.011","volume":"1812","author":"E Kabashi","year":"2011","unstructured":"Kabashi E, Brustein E, Champagne N, Drapeau P (2011) Zebrafish models for the functional genomics of neurogenetic disorders. Biochim Biophys Acta 1812:335\u2013345","journal-title":"Biochim Biophys Acta"},{"key":"5_CR93","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1007\/s11910-011-0182-2","volume":"11","author":"Y Xi","year":"2011","unstructured":"Xi Y, Noble S, Ekker M (2011) Modeling neurodegeneration in zebrafish. Curr Neurol Neurosci Rep 11:274\u2013282","journal-title":"Curr Neurol Neurosci Rep"},{"key":"5_CR94","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1002\/dneu.20918","volume":"72","author":"C Kizil","year":"2012","unstructured":"Kizil C, Kaslin J, Kroehne V, Brand M (2012) Adult neurogenesis and brain regeneration in zebrafish. Dev Neurobiol 72:429\u2013461","journal-title":"Dev Neurobiol"},{"key":"5_CR95","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.pneurobio.2014.03.001","volume":"118","author":"PJ Babin","year":"2014","unstructured":"Babin PJ, Goizet C, Raldua D (2014) Zebrafish models of human motor neuron diseases: advantages and limitations. Prog Neurobiol 118:36\u201358","journal-title":"Prog Neurobiol"},{"key":"5_CR96","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1038\/nature12111","volume":"496","author":"K Howe","year":"2013","unstructured":"Howe K, Clark MD, Torroja CF et al (2013) The zebrafish reference genome sequence and its relationship to the human genome. Nature 496:498\u2013503","journal-title":"Nature"},{"key":"5_CR97","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1242\/jcs.140194","volume":"127","author":"AM Vacaru","year":"2014","unstructured":"Vacaru AM, Unlu G, Spitzner M et al (2014) In vivo cell biology in zebrafish\u00a0\u2013 providing insights into vertebrate development and disease. J Cell Sci 127:485\u2013495","journal-title":"J Cell Sci"},{"key":"5_CR98","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1002\/glia.22780","volume":"63","author":"N Oosterhof","year":"2015","unstructured":"Oosterhof N, Boddeke E, van Ham TJ (2015) Immune cell dynamics in the CNS: learning from the zebrafish. Glia 63:719\u2013735","journal-title":"Glia"},{"key":"5_CR99","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1016\/j.neubiorev.2014.06.013","volume":"45","author":"S Das","year":"2014","unstructured":"Das S, Rajanikant GK (2014) Huntington disease: can a zebrafish trail leave more than a ripple? Neurosci Biobehav Rev 45:258\u2013261","journal-title":"Neurosci Biobehav Rev"},{"key":"5_CR100","doi-asserted-by":"crossref","first-page":"9152","DOI":"10.1523\/JNEUROSCI.3001-05.2005","volume":"25","author":"VM Miller","year":"2005","unstructured":"Miller VM, Nelson RF, Gouvion CM et al (2005) CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J Neurosci 25:9152\u20139161","journal-title":"J Neurosci"},{"key":"5_CR101","doi-asserted-by":"crossref","first-page":"9195","DOI":"10.1074\/jbc.M607865200","volume":"282","author":"NW Schiffer","year":"2007","unstructured":"Schiffer NW, Broadley SA, Hirschberger T et al (2007) Identification of anti-prion compounds as efficient inhibitors of polyglutamine protein aggregation in a zebrafish model. J Biol Chem 282:9195\u20139203","journal-title":"J Biol Chem"},{"key":"5_CR102","doi-asserted-by":"crossref","first-page":"1905","DOI":"10.1093\/hmg\/ddm138","volume":"16","author":"AL Lumsden","year":"2007","unstructured":"Lumsden AL, Henshall TL, Dayan S et al (2007) Huntingtin-deficient zebrafish exhibit defects in iron utilization and development. Hum Mol Genet 16:1905\u20131920","journal-title":"Hum Mol Genet"},{"key":"5_CR103","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1038\/nn.3080","volume":"15","author":"V Lo Sardo","year":"2012","unstructured":"Lo Sardo V, Zuccato C, Gaudenzi G et al (2012) An evolutionary recent neuroepithelial cell adhesion function of huntingtin implicates ADAM10-Ncadherin. Nat Neurosci 15:713\u2013721","journal-title":"Nat Neurosci"},{"key":"5_CR104","doi-asserted-by":"crossref","first-page":"3224","DOI":"10.1073\/pnas.0400243101","volume":"101","author":"WC Lee","year":"2004","unstructured":"Lee WC, Yoshihara M, Littleton JT (2004) Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington\u2019s disease. Proc Natl Acad Sci U S A 101:3224\u20133229","journal-title":"Proc Natl Acad Sci U S A"},{"key":"5_CR105","doi-asserted-by":"crossref","first-page":"2912","DOI":"10.1093\/hmg\/dds117","volume":"21","author":"JR Steinert","year":"2012","unstructured":"Steinert JR, Campesan S, Richards P et al (2012) Rab11 rescues synaptic dysfunction and behavioural deficits in a Drosophila model of Huntington\u2019s disease. Hum Mol Genet 21:2912\u20132922","journal-title":"Hum Mol Genet"},{"key":"5_CR106","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0068775","volume":"8","author":"Y Arribat","year":"2013","unstructured":"Arribat Y, Bonneaud N, Talmat-Amar Y et al (2013) A huntingtin peptide inhibits polyQ-huntingtin associated defects. PLoS One 8:e68775","journal-title":"PLoS One"},{"key":"5_CR107","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/j.conb.2011.11.008","volume":"22","author":"A Gomez-Marin","year":"2012","unstructured":"Gomez-Marin A, Louis M (2012) Active sensation during orientation behavior in the Drosophila larva: more sense than luck. Curr Opin Neurobiol 22:208\u2013215","journal-title":"Curr Opin Neurobiol"},{"key":"5_CR108","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1038\/ncomms1455","volume":"2","author":"A Gomez-Marin","year":"2011","unstructured":"Gomez-Marin A, Stephens GJ, Louis M (2011) Active sampling and decision making in Drosophila chemotaxis. Nat Commun 2:441","journal-title":"Nat Commun"},{"key":"5_CR109","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1007\/978-1-62703-438-8_3","volume":"1017","author":"W Song","year":"2013","unstructured":"Song W, Smith MR, Syed A et al (2013) Morphometric analysis of Huntington\u2019s disease neurodegeneration in Drosophila. Methods Mol Biol 1017:41\u201357","journal-title":"Methods Mol Biol"},{"key":"5_CR110","doi-asserted-by":"crossref","first-page":"3113","DOI":"10.1523\/JNEUROSCI.21-09-03113.2001","volume":"21","author":"KD Bodily","year":"2001","unstructured":"Bodily KD, Morrison CM, Renden RB, Broadie K (2001) A novel member of the Ig superfamily, turtle, is a CNS-specific protein required for coordinated motor control. J Neurosci 21:3113\u20133125","journal-title":"J Neurosci"},{"key":"5_CR111","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1016\/j.mcn.2008.01.003","volume":"37","author":"L Pan","year":"2008","unstructured":"Pan L, Woodruff E, Liang P, Broadie K (2008) Mechanistic relationships between Drosophila fragile X mental retardation protein and metabotropic glutamate receptor. A signaling. Mol Cell Neurosci 37:747\u2013760","journal-title":"Mol Cell Neurosci"},{"key":"5_CR112","volume":"9","author":"R Shiraishi","year":"2014","unstructured":"Shiraishi R, Tamura T, Sone M, Okazawa H (2014) Systematic analysis of fly models with multiple drivers reveals different effects of ataxin-1 and huntingtin in neuron subtype-specific expression. PLoS One 9:e116567","journal-title":"PLoS One"},{"key":"5_CR113","doi-asserted-by":"crossref","first-page":"2510","DOI":"10.1093\/hmg\/ddr150","volume":"20","author":"NA Lanson","year":"2011","unstructured":"Lanson NA, Maltare A, King H et al (2011) A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. Hum Mol Genet 20:2510\u20132523","journal-title":"Hum Mol Genet"},{"key":"5_CR114","doi-asserted-by":"crossref","first-page":"3312","DOI":"10.1073\/pnas.0611511104","volume":"104","author":"J Bischof","year":"2007","unstructured":"Bischof J, Maeda RK, Hediger M et al (2007) An optimized transgenesis system for Drosophila using germ-line-specific phiC31 integrases. Proc Natl Acad Sci U S A 104:3312\u20133317","journal-title":"Proc Natl Acad Sci U S A"}],"container-title":["Methods in Molecular Biology","Huntington\u2019s Disease"],"original-title":[],"link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/978-1-4939-7825-0_5","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T20:17:26Z","timestamp":1661372246000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/978-1-4939-7825-0_5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018]]},"ISBN":["9781493978243","9781493978250"],"references-count":114,"URL":"https:\/\/doi.org\/10.1007\/978-1-4939-7825-0_5","relation":{},"ISSN":["1064-3745","1940-6029"],"issn-type":[{"value":"1064-3745","type":"print"},{"value":"1940-6029","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018]]}}}