{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T05:54:43Z","timestamp":1792907683228,"version":"4.5.0"},"reference-count":61,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2023,8,8]],"date-time":"2023-08-08T00:00:00Z","timestamp":1691452800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS086778"],"award-info":[{"award-number":["NS086778"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,9,30]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Spinocerebellar Ataxia Type 17 (SCA17) is the most recently identified member of the polyglutamine (polyQ) family of disorders, resulting from abnormal CAG\/CAA expansion in the TATA box-binding protein (TBP), an initiation factor essential for of all eukaryotic transcription. A largely autosomal dominant inherited disease, SCA17, is unique in both its heterogeneous clinical presentation and low incidence of genetic anticipation, the phenomenon in which subsequent generations inherit longer polyQ expansions that yield earlier and more severe symptom onset. Like other polyQ disease family members, SCA17 patients experience progressive ataxia and dementia, and treatments are limited to preventing symptoms and increasing quality of life. Here, we report 2 new Drosophila models that express human TBP with polyQ repeats in either wild-type or SCA17 patient range. We find that TBP expression has age- and tissue-specific effects on neurodegeneration, with polyQ-expanded SCA17 protein expression generally having more severe effects. In addition, SCA17 model flies accumulate more aggregation-prone TBP, with a greater proportion localizing to the nucleus. These new lines provide a new resource for the biochemical characterization of SCA17 pathology and the future identification of therapeutic targets.<\/jats:p>","DOI":"10.1093\/g3journal\/jkad180","type":"journal-article","created":{"date-parts":[[2023,8,8]],"date-time":"2023-08-08T07:45:12Z","timestamp":1691480712000},"source":"Crossref","is-referenced-by-count":11,"title":["Phenotypic defects from the expression of wild-type and pathogenic TATA-binding proteins in new <i>Drosophila<\/i> models of Spinocerebellar Ataxia Type 17"],"prefix":"10.1093","volume":"13","author":[{"given":"Nikhil","family":"Patel","sequence":"first","affiliation":[{"name":"Department of Pharmacology, School of Medicine, Wayne State University , Detroit, MI 48201 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadir","family":"Alam","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, School of Medicine, Wayne State University , Detroit, MI 48201 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kozeta","family":"Libohova","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, School of Medicine, Wayne State University , Detroit, MI 48201 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan","family":"Dulay","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, School of Medicine, Wayne State University , Detroit, MI 48201 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sokol V","family":"Todi","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, School of Medicine, Wayne State University , Detroit, MI 48201 ,","place":["USA"]},{"name":"Department of Neurology, School of Medicine, Wayne State University , Detroit, MI 48201 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alyson","family":"Sujkowski","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, School of Medicine, Wayne State University , Detroit, MI 48201 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,8,8]]},"reference":[{"issue":"1","key":"2025020615422692900_jkad180-B1","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.parkreldis.2009.06.006","article-title":"Huntington's disease-like and ataxia syndromes: identification of a family with a de novo SCA17\/TBP mutation","volume":"16","author":"Bech","year":"2010","journal-title":"Parkinsonism Relat Disord"},{"issue":"10","key":"2025020615422692900_jkad180-B2","doi-asserted-by":"publisher","first-page":"2096","DOI":"10.1002\/jnr.24684","article-title":"Degron capability of the hydrophobic C-terminus of the polyglutamine disease protein, ataxin-3","volume":"98","author":"Blount","year":"2020","journal-title":"J Neurosci Res"},{"issue":"1","key":"2025020615422692900_jkad180-B3","doi-asserted-by":"publisher","first-page":"4638","DOI":"10.1038\/ncomms5638","article-title":"Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23","volume":"5","author":"Blount","year":"2014","journal-title":"Nat Commun"},{"key":"2025020615422692900_jkad180-B4","first-page":"350a","article-title":"Using Drosophila to model human neurodegenerative disease","volume":"10","author":"Bonini","year":"1999","journal-title":"Mol Biol Cell"},{"issue":"1","key":"2025020615422692900_jkad180-B5","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1146\/annurev.neuro.26.041002.131425","article-title":"Human neurodegenerative disease modeling using Drosophila","volume":"26","author":"Bonini","year":"2003","journal-title":"Annu Rev Neurosci"},{"issue":"2","key":"2025020615422692900_jkad180-B6","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1242\/dev.118.2.401","article-title":"Targeted gene-expression as a means of altering cell fates and generating dominant phenotypes","volume":"118","author":"Brand","year":"1993","journal-title":"Development"},{"issue":"2","key":"2025020615422692900_jkad180-B7","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1007\/s13311-018-00696-y","article-title":"Genetics, mechanisms, and therapeutic progress in polyglutamine spinocerebellar ataxias","volume":"16","author":"Buijsen","year":"2019","journal-title":"Neurotherapeutics"},{"issue":"1","key":"2025020615422692900_jkad180-B8","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1146\/annurev.bi.65.070196.004005","article-title":"Biochemistry and structural biology of transcription factor IID (TFIID)","volume":"65","author":"Burley","year":"1996","journal-title":"Annu Rev Biochem"},{"issue":"9","key":"2025020615422692900_jkad180-B9","doi-asserted-by":"publisher","first-page":"1100","DOI":"10.1002\/jnr.23395","article-title":"Using membrane-targeted green fluorescent protein to monitor neurotoxic protein-dependent degeneration of Drosophila eyes","volume":"92","author":"Burr","year":"2014","journal-title":"J Neurosci Res"},{"key":"2025020615422692900_jkad180-B10","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1016\/j.brainres.2014.09.064","article-title":"A fruitful endeavor: modeling ALS in the fruit fly","volume":"1607","author":"Casci","year":"2015","journal-title":"Brain Res"},{"issue":"4","key":"2025020615422692900_jkad180-B11","doi-asserted-by":"publisher","first-page":"1260","DOI":"10.3390\/ijms21041260","article-title":"Growth and maturation in development: a fly's perspective","volume":"21","author":"Delanoue","year":"2020","journal-title":"Int J Mol Sci"},{"issue":"12","key":"2025020615422692900_jkad180-B12","doi-asserted-by":"publisher","first-page":"1519","DOI":"10.1038\/nn2011","article-title":"Polyglutamine domain modulates the TBP\u2013TFIIB interaction: implications for its normal function and neurodegeneration","volume":"10","author":"Friedman","year":"2007","journal-title":"Nat Neurosci"},{"issue":"13","key":"2025020615422692900_jkad180-B13","doi-asserted-by":"publisher","first-page":"8283","DOI":"10.1074\/jbc.M709674200","article-title":"Polyglutamine expansion reduces the association of TATA-binding protein with DNA and induces DNA binding-independent neurotoxicity","volume":"283","author":"Friedman","year":"2008","journal-title":"J Biol Chem"},{"issue":"10","key":"2025020615422692900_jkad180-B14","doi-asserted-by":"publisher","first-page":"1939","DOI":"10.1093\/brain\/124.10.1939","article-title":"CAG repeat expansion in the TATA box-binding protein gene causes autosomal dominant cerebellar ataxia","volume":"124","author":"Fujigasaki","year":"2001","journal-title":"Brain"},{"issue":"45","key":"2025020615422692900_jkad180-B15","doi-asserted-by":"publisher","first-page":"16269","DOI":"10.1523\/JNEUROSCI.4000-11.2011","article-title":"Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous Bergmann glia degeneration","volume":"31","author":"Furrer","year":"2011","journal-title":"J Neurosci"},{"issue":"2","key":"2025020615422692900_jkad180-B16","doi-asserted-by":"publisher","first-page":"215","DOI":"10.1038\/sj.ejhg.5201954","article-title":"Instability of expanded CAG\/CAA repeats in spinocerebellar ataxia type 17","volume":"16","author":"Gao","year":"2008","journal-title":"Eur J Hum Genet"},{"issue":"6","key":"2025020615422692900_jkad180-B17","doi-asserted-by":"publisher","first-page":"650","DOI":"10.1001\/jamaneurol.2019.0423","article-title":"Prevalence of carriers of intermediate and pathological polyglutamine disease-associated alleles among large population-based cohorts","volume":"76","author":"Gardiner","year":"2019","journal-title":"JAMA Neurol"},{"issue":"4","key":"2025020615422692900_jkad180-B18","doi-asserted-by":"publisher","first-page":"1775","DOI":"10.1093\/genetics\/166.4.1775","article-title":"Construction of transgenic Drosophila by using the site-specific integrase from phage phi C31","volume":"166","author":"Groth","year":"2004","journal-title":"Genetics"},{"issue":"25","key":"2025020615422692900_jkad180-B19","doi-asserted-by":"publisher","first-page":"6878","DOI":"10.1093\/hmg\/ddu410","article-title":"Deactivation of TBP contributes to SCA17 pathogenesis","volume":"23","author":"Hsu","year":"2014","journal-title":"Hum Mol Genet"},{"issue":"7","key":"2025020615422692900_jkad180-B20","doi-asserted-by":"publisher","first-page":"1943","DOI":"10.1093\/brain\/awr146","article-title":"Neuronal expression of TATA box-binding protein containing expanded polyglutamine in knock-in mice reduces chaperone protein response by impairing the function of nuclear factor-Y transcription factor","volume":"134","author":"Huang","year":"2011","journal-title":"Brain"},{"issue":"1","key":"2025020615422692900_jkad180-B21","doi-asserted-by":"publisher","first-page":"196","DOI":"10.1016\/j.celrep.2015.08.060","article-title":"Large polyglutamine repeats cause muscle degeneration in SCA17 mice","volume":"13","author":"Huang","year":"2015","journal-title":"Cell Rep"},{"key":"2025020615422692900_jkad180-B22","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2019.104535","article-title":"Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3","volume":"132","author":"Johnson","year":"2019","journal-title":"Neurobiol Dis"},{"issue":"7","key":"2025020615422692900_jkad180-B23","doi-asserted-by":"publisher","first-page":"1223","DOI":"10.3390\/cells11071223","article-title":"Drosophila as a model of unconventional translation in Spinocerebellar Ataxia Type 3","volume":"11","author":"Johnson","year":"2022","journal-title":"Cells"},{"key":"2025020615422692900_jkad180-B24","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2022.974167","article-title":"A survey of protein interactions and posttranslational modifications that influence the polyglutamine diseases","volume":"15","author":"Johnson","year":"2022","journal-title":"Front Mol Neurosci"},{"issue":"11","key":"2025020615422692900_jkad180-B25","doi-asserted-by":"publisher","first-page":"2047","DOI":"10.1093\/hmg\/8.11.2047","article-title":"A neurological disease caused by an expanded CAG trinucleotide repeat in the TATA-binding protein gene: a new polyglutamine disease?","volume":"8","author":"Koide","year":"1999","journal-title":"Hum Mol Genet"},{"issue":"5","key":"2025020615422692900_jkad180-B26","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1016\/S0896-6273(03)00328-3","article-title":"Polyglutamines placed into context","volume":"38","author":"La Spada","year":"2003","journal-title":"Neuron"},{"issue":"1","key":"2025020615422692900_jkad180-B27","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1146\/annurev-pathmechdis-012418-012857","article-title":"Polyglutamine repeats in neurodegenerative diseases","volume":"14","author":"Lieberman","year":"2019","journal-title":"Annu Rev Pathol"},{"issue":"4","key":"2025020615422692900_jkad180-B28","doi-asserted-by":"publisher","first-page":"1097","DOI":"10.1007\/s13311-019-00762-z","article-title":"Molecular mechanisms and therapeutics for SCA17","volume":"16","author":"Liu","year":"2019","journal-title":"Neurotherapeutics"},{"issue":"1","key":"2025020615422692900_jkad180-B29","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1016\/j.gim.2021.08.003","article-title":"Digenic inheritance of STUB1 variants and TBP polyglutamine expansions explains the incomplete penetrance of SCA17 and SCA48","volume":"24","author":"Magri","year":"2022","journal-title":"Genet Med"},{"issue":"10","key":"2025020615422692900_jkad180-B30","doi-asserted-by":"publisher","first-page":"1441","DOI":"10.1212\/01.WNL.0000094123.09098.A0","article-title":"Intergenerational instability and marked anticipation in SCA-17","volume":"61","author":"Maltecca","year":"2003","journal-title":"Neurology"},{"issue":"4","key":"2025020615422692900_jkad180-B31","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1038\/ng.101","article-title":"Exploiting position effects and the gypsy retrovirus insulator to engineer precisely expressed transgenes","volume":"40","author":"Markstein","year":"2008","journal-title":"Nat Genet"},{"issue":"5595","key":"2025020615422692900_jkad180-B32","doi-asserted-by":"publisher","first-page":"1036","DOI":"10.1126\/science.1076327","article-title":"RNA polymerase II transcription in murine cells lacking the TATA binding protein","volume":"298","author":"Martianov","year":"2002","journal-title":"Science"},{"issue":"2\u20135","key":"2025020615422692900_jkad180-B33","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/S0960-0760(03)00231-0","article-title":"Androgen receptor functions from reverse genetic models","volume":"85","author":"Matsumoto","year":"2003","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"1","key":"2025020615422692900_jkad180-B34","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1093\/hmg\/ddr439","article-title":"Protein interacting with C kinase (PICK1) is a suppressor of spinocerebellar ataxia 3-associated neurodegeneration in Drosophila","volume":"21","author":"McGurk","year":"2012","journal-title":"Hum Mol Genet"},{"key":"2025020615422692900_jkad180-B35","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1007\/978-1-4939-8669-9_22","article-title":"Analysis of the Drosophila compound eye with light and electron microscopy","volume":"1834","author":"Mishra","year":"2019","journal-title":"Methods Mol Biol"},{"issue":"14","key":"2025020615422692900_jkad180-B36","doi-asserted-by":"publisher","first-page":"1441","DOI":"10.1093\/hmg\/10.14.1441","article-title":"SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein","volume":"10","author":"Nakamura","year":"2001","journal-title":"Hum Mol Genet"},{"issue":"1","key":"2025020615422692900_jkad180-B37","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1038\/nmeth1146","article-title":"Vector and parameters for targeted transgenic RNA interference in Drosophila melanogaster","volume":"5","author":"Ni","year":"2008","journal-title":"Nat Methods"},{"issue":"1","key":"2025020615422692900_jkad180-B38","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/s40673-018-0086-x","article-title":"Spinocerebellar ataxia 17: full phenotype in a 41 CAG\/CAA repeats carrier","volume":"5","author":"Origone","year":"2018","journal-title":"Cerebellum Ataxias"},{"key":"2025020615422692900_jkad180-B39","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1016\/j.neurobiolaging.2018.07.022","article-title":"Beta-sheet assembly of Tau and neurodegeneration in Drosophila melanogaster","volume":"72","author":"Passarella","year":"2018","journal-title":"Neurobiol Aging"},{"issue":"10","key":"2025020615422692900_jkad180-B40","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1038\/nrn.2017.92","article-title":"Polyglutamine spinocerebellar ataxias\u2014from genes to potential treatments","volume":"18","author":"Paulson","year":"2017","journal-title":"Nat Rev Neurosci"},{"key":"2025020615422692900_jkad180-B41","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2022.1112688","article-title":"Ubiquitin-binding site 1 of pathogenic ataxin-3 regulates its toxicity in Drosophila models of Spinocerebellar Ataxia Type 3","volume":"16","author":"Prifti","year":"2023","journal-title":"Front Neurosci"},{"issue":"17","key":"2025020615422692900_jkad180-B42","doi-asserted-by":"publisher","first-page":"3424","DOI":"10.1093\/hmg\/ddr251","article-title":"A Drosophila model of the neurodegenerative disease SCA17 reveals a role of RBP-J\/Su(H) in modulating the pathological outcome","volume":"20","author":"Ren","year":"2011","journal-title":"Hum Mol Genet"},{"key":"2025020615422692900_jkad180-B43","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/j.nbd.2018.04.013","article-title":"Toxicity and aggregation of the polyglutamine disease protein, ataxin-3 is regulated by its binding to VCP\/p97 in Drosophila melanogaster","volume":"116","author":"Ristic","year":"2018","journal-title":"Neurobiol Dis"},{"issue":"1\u20132","key":"2025020615422692900_jkad180-B44","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1002\/gene.10133","article-title":"Conditional expression of UAS-transgenes in the adult eye with a new gene-switch vector system","volume":"34","author":"Roman","year":"2002","journal-title":"Genesis"},{"issue":"8","key":"2025020615422692900_jkad180-B45","doi-asserted-by":"publisher","first-page":"1604","DOI":"10.1523\/JNEUROSCI.1954-20.2021","article-title":"Impaired oligodendrocyte maturation is an early feature in SCA3 disease pathogenesis","volume":"42","author":"Schuster","year":"2022","journal-title":"J Neurosci"},{"key":"2025020615422692900_jkad180-B46","doi-asserted-by":"publisher","DOI":"10.3791\/61363","article-title":"Visualizing synaptic degeneration in adult Drosophila in association with neurodegeneration","author":"Sidisky","year":"2020","journal-title":"J Vis Exp"},{"issue":"16","key":"2025020615422692900_jkad180-B47","doi-asserted-by":"publisher","first-page":"8930","DOI":"10.3390\/ijms22168930","article-title":"Application of fluorescent proteins for functional dissection of the Drosophila visual system","volume":"22","author":"Smylla","year":"2021","journal-title":"Int J Mol Sci"},{"issue":"3","key":"2025020615422692900_jkad180-B48","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1038\/nrg751","article-title":"The art and design of genetic screens: Drosophila melanogaster","volume":"3","author":"St Johnston","year":"2002","journal-title":"Nat Rev Genet"},{"key":"2025020615422692900_jkad180-B49","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.75389","article-title":"Endurance exercise ameliorates phenotypes in Drosophila models of spinocerebellar ataxias","volume":"11","author":"Sujkowski","year":"2022","journal-title":"Elife"},{"issue":"8","key":"2025020615422692900_jkad180-B50","doi-asserted-by":"publisher","first-page":"1419","DOI":"10.1093\/hmg\/ddx039","article-title":"Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3","volume":"26","author":"Sutton","year":"2017","journal-title":"Hum Mol Genet"},{"key":"2025020615422692900_jkad180-B51","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/B978-012369509-3.50019-6","volume-title":"Molecular Neurology","author":"Todi","year":"2007"},{"key":"2025020615422692900_jkad180-B52","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/B978-012369509-3.50019-6","volume-title":"Molecular Neurology","author":"Todi","year":"2007"},{"issue":"6","key":"2025020615422692900_jkad180-B53","doi-asserted-by":"publisher","first-page":"e95","DOI":"10.1371\/journal.pgen.0030095","article-title":"No influence of Indy on lifespan in Drosophila after correction for genetic and cytoplasmic background effects","volume":"3","author":"Toivonen","year":"2007","journal-title":"PLoS Genet"},{"key":"2025020615422692900_jkad180-B54","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1007\/978-3-319-71779-1_10","article-title":"Spinocerebellar Ataxia Type 17 (SCA17)","volume":"1049","author":"Toyoshima","year":"2018","journal-title":"Adv Exp Med Biol"},{"issue":"48","key":"2025020615422692900_jkad180-B55","doi-asserted-by":"publisher","first-page":"34460","DOI":"10.1074\/jbc.M113.513903","article-title":"Ubiquitination regulates the neuroprotective function of the deubiquitinase Ataxin-3 in vivo","volume":"288","author":"Tsou","year":"2013","journal-title":"J Biol Chem"},{"issue":"15","key":"2025020615422692900_jkad180-B56","doi-asserted-by":"publisher","first-page":"4385","DOI":"10.1093\/hmg\/ddv174","article-title":"DnaJ-1 and karyopherin alpha3 suppress degeneration in a new Drosophila model of Spinocerebellar Ataxia Type 6","volume":"24","author":"Tsou","year":"2015","journal-title":"Hum Mol Genet"},{"issue":"12","key":"2025020615422692900_jkad180-B57","doi-asserted-by":"publisher","first-page":"1770","DOI":"10.1242\/bio.021667","article-title":"Polyglutamine length-dependent toxicity from alpha1ACT in Drosophila models of spinocerebellar ataxia type 6","volume":"5","author":"Tsou","year":"2016","journal-title":"Biol Open"},{"key":"2025020615422692900_jkad180-B58","doi-asserted-by":"publisher","first-page":"331","DOI":"10.3389\/fnins.2019.00331","article-title":"Dissecting motor neuron disease with Drosophila melanogaster","volume":"13","author":"Walters","year":"2019","journal-title":"Front Neurosci"},{"issue":"38","key":"2025020615422692900_jkad180-B59","doi-asserted-by":"publisher","first-page":"9101","DOI":"10.1523\/JNEUROSCI.0111-17.2017","article-title":"Synergistic toxicity of polyglutamine-expanded TATA-binding protein in glia and neuronal cells: therapeutic implications for Spinocerebellar Ataxia 17","volume":"37","author":"Yang","year":"2017","journal-title":"J Neurosci"},{"issue":"1","key":"2025020615422692900_jkad180-B60","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1146\/annurev.neuro.23.1.217","article-title":"Glutamine repeats and neurodegeneration","volume":"23","author":"Zoghbi","year":"2000","journal-title":"Annu Rev Neurosci"},{"issue":"12","key":"2025020615422692900_jkad180-B61","doi-asserted-by":"publisher","first-page":"7425","DOI":"10.1074\/jbc.R800041200","article-title":"Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1","volume":"284","author":"Zoghbi","year":"2009","journal-title":"J Biol Chem"}],"container-title":["G3: Genes, Genomes, Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/g3journal\/advance-article-pdf\/doi\/10.1093\/g3journal\/jkad180\/51299936\/jkad180.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/g3journal\/article-pdf\/13\/10\/jkad180\/56715530\/jkad180.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/g3journal\/article-pdf\/13\/10\/jkad180\/56715530\/jkad180.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,6]],"date-time":"2025-02-06T15:42:47Z","timestamp":1738856567000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/g3journal\/article\/doi\/10.1093\/g3journal\/jkad180\/7238619"}},"subtitle":[],"editor":[{"given":"J","family":"Tennessen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2023,8,8]]},"references-count":61,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2023,8,8]]},"published-print":{"date-parts":[[2023,9,30]]}},"URL":"https:\/\/doi.org\/10.1093\/g3journal\/jkad180","relation":{},"ISSN":["2160-1836"],"issn-type":[{"value":"2160-1836","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,10]]},"published":{"date-parts":[[2023,8,8]]},"article-number":"jkad180"}}