{"id":"https://openalex.org/W1885640155","doi":"https://doi.org/10.1371/journal.pone.0141610","title":"Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease","display_name":"Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease","publication_year":2015,"publication_date":"2015-10-27","ids":{"openalex":"https://openalex.org/W1885640155","doi":"https://doi.org/10.1371/journal.pone.0141610","mag":"1885640155","pmid":"https://pubmed.ncbi.nlm.nih.gov/26505994"},"language":"en","primary_location":{"id":"doi:10.1371/journal.pone.0141610","doi":"https://doi.org/10.1371/journal.pone.0141610","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0141610","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0141610&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLOS ONE","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0141610&type=printable","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5106669727","display_name":"Sofia Esteves","orcid":"https://orcid.org/0000-0001-8249-9690","observed_orcids":["https://orcid.org/0000-0001-8249-9690"]},"institutions":[{"id":"https://openalex.org/I99682543","display_name":"University of Minho","ror":"https://ror.org/037wpkx04","country_code":"PT","type":"education","lineage":["https://openalex.org/I99682543"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Sofia Esteves","raw_affiliation_strings":["Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":["https://openalex.org/I99682543"]},{"raw_affiliation_string":"ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":[]},{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal","institution_ids":["https://openalex.org/I99682543"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5079933113","display_name":"Sara Duarte\u2010Silva","orcid":"https://orcid.org/0000-0001-9680-4027","observed_orcids":["https://orcid.org/0000-0001-9680-4027"]},"institutions":[{"id":"https://openalex.org/I99682543","display_name":"University of Minho","ror":"https://ror.org/037wpkx04","country_code":"PT","type":"education","lineage":["https://openalex.org/I99682543"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Sara Duarte-Silva","raw_affiliation_strings":["Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":["https://openalex.org/I99682543"]},{"raw_affiliation_string":"ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":[]},{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal","institution_ids":["https://openalex.org/I99682543"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5078998438","display_name":"Luana Naia","orcid":"https://orcid.org/0000-0002-0886-4634","observed_orcids":["https://orcid.org/0000-0002-0886-4634"]},"institutions":[{"id":"https://openalex.org/I4394709155","display_name":"Centro de Neuroci\u00eancias e Biologia Celular","ror":"https://ror.org/01nzkd566","country_code":"PT","type":"facility","lineage":["https://openalex.org/I4394709155","https://openalex.org/I76903346"]},{"id":"https://openalex.org/I76903346","display_name":"University of Coimbra","ror":"https://ror.org/04z8k9a98","country_code":"PT","type":"education","lineage":["https://openalex.org/I76903346"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Luana Naia","raw_affiliation_strings":["CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Coimbra, Portugal","CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal","Faculty of Medicine, University of Coimbra, Coimbra, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Coimbra, Portugal","institution_ids":["https://openalex.org/I76903346"]},{"raw_affiliation_string":"CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal","institution_ids":["https://openalex.org/I4394709155","https://openalex.org/I76903346"]},{"raw_affiliation_string":"Faculty of Medicine, University of Coimbra, Coimbra, Portugal","institution_ids":["https://openalex.org/I76903346"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5064849094","display_name":"Andreia Neves\u2010Carvalho","orcid":"https://orcid.org/0000-0002-5710-0040","observed_orcids":["https://orcid.org/0000-0002-5710-0040"]},"institutions":[{"id":"https://openalex.org/I99682543","display_name":"University of Minho","ror":"https://ror.org/037wpkx04","country_code":"PT","type":"education","lineage":["https://openalex.org/I99682543"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Andreia Neves-Carvalho","raw_affiliation_strings":["Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":["https://openalex.org/I99682543"]},{"raw_affiliation_string":"ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":[]},{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal","institution_ids":["https://openalex.org/I99682543"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5035750485","display_name":"Andreia Teixeira\u2010Castro","orcid":"https://orcid.org/0000-0003-4999-5996","observed_orcids":["https://orcid.org/0000-0003-4999-5996"]},"institutions":[{"id":"https://openalex.org/I99682543","display_name":"University of Minho","ror":"https://ror.org/037wpkx04","country_code":"PT","type":"education","lineage":["https://openalex.org/I99682543"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Andreia Teixeira-Castro","raw_affiliation_strings":["Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":["https://openalex.org/I99682543"]},{"raw_affiliation_string":"ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":[]},{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal","institution_ids":["https://openalex.org/I99682543"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5043027619","display_name":"Ana Cristina Rego","orcid":"https://orcid.org/0000-0003-0700-3776","observed_orcids":["https://orcid.org/0000-0003-0700-3776"]},"institutions":[{"id":"https://openalex.org/I4394709155","display_name":"Centro de Neuroci\u00eancias e Biologia Celular","ror":"https://ror.org/01nzkd566","country_code":"PT","type":"facility","lineage":["https://openalex.org/I4394709155","https://openalex.org/I76903346"]},{"id":"https://openalex.org/I76903346","display_name":"University of Coimbra","ror":"https://ror.org/04z8k9a98","country_code":"PT","type":"education","lineage":["https://openalex.org/I76903346"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Ana Cristina Rego","raw_affiliation_strings":["CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Coimbra, Portugal","CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal","Faculty of Medicine, University of Coimbra, Coimbra, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Coimbra, Portugal","institution_ids":["https://openalex.org/I76903346"]},{"raw_affiliation_string":"CNC-Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal","institution_ids":["https://openalex.org/I4394709155","https://openalex.org/I76903346"]},{"raw_affiliation_string":"Faculty of Medicine, University of Coimbra, Coimbra, Portugal","institution_ids":["https://openalex.org/I76903346"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5078747573","display_name":"Anabela Silva\u2010Fernandes","orcid":"https://orcid.org/0000-0002-1187-7815","observed_orcids":["https://orcid.org/0000-0002-1187-7815"]},"institutions":[{"id":"https://openalex.org/I99682543","display_name":"University of Minho","ror":"https://ror.org/037wpkx04","country_code":"PT","type":"education","lineage":["https://openalex.org/I99682543"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Anabela Silva-Fernandes","raw_affiliation_strings":["Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":["https://openalex.org/I99682543"]},{"raw_affiliation_string":"ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":[]},{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal","institution_ids":["https://openalex.org/I99682543"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5000402803","display_name":"Patr\u0131\u0301cia Maciel","orcid":"https://orcid.org/0000-0002-0920-6350","observed_orcids":["https://orcid.org/0000-0002-0920-6350"]},"institutions":[{"id":"https://openalex.org/I99682543","display_name":"University of Minho","ror":"https://ror.org/037wpkx04","country_code":"PT","type":"education","lineage":["https://openalex.org/I99682543"]}],"countries":["PT"],"is_corresponding":true,"raw_author_name":"Patr\u00edcia Maciel","raw_affiliation_strings":["Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga, Portugal; ICVS/3Bs-PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":["https://openalex.org/I99682543"]},{"raw_affiliation_string":"ICVS/3Bs\u2014PT Government Associate Laboratory, Braga/Guimar\u00e3es, Portugal","institution_ids":[]},{"raw_affiliation_string":"Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710\u2013057 Braga, Portugal","institution_ids":["https://openalex.org/I99682543"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":3,"corresponding_author_ids":["https://openalex.org/A5000402803"],"corresponding_institution_ids":["https://openalex.org/I99682543"],"apc_list":{"value":2382,"currency":"USD","value_usd":2382},"apc_paid":{"value":2382,"currency":"USD","value_usd":2382},"fwci":1.5187,"has_fulltext":true,"cited_by_count":26,"citation_normalized_percentile":{"value":0.79270908,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":99},"biblio":{"volume":"10","issue":"10","first_page":"e0141610","last_page":"e0141610"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10949","display_name":"Genetic Neurodegenerative Diseases","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/2804","display_name":"Cellular and Molecular Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T10949","display_name":"Genetic Neurodegenerative Diseases","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/2804","display_name":"Cellular and Molecular Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10855","display_name":"Amyotrophic Lateral Sclerosis Research","score":0.00019999999494757503,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Neurology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11772","display_name":"Genetics and Neurodevelopmental Disorders","score":0.00019999999494757503,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/spinocerebellar-ataxia-type-3","display_name":"spinocerebellar ataxia type 3","score":0.9980000257492065},{"id":"https://openalex.org/keywords/valproic-acid","display_name":"valproic acid","score":0.9959999918937683},{"id":"https://openalex.org/keywords/ataxin-3","display_name":"ataxin-3","score":0.9300000071525574},{"id":"https://openalex.org/keywords/cag-repeat-expansion","display_name":"CAG repeat expansion","score":0.9919999837875366},{"id":"https://openalex.org/keywords/neurodegeneration","display_name":"neurodegeneration","score":0.6389999985694885},{"id":"https://openalex.org/keywords/neuroprotection","display_name":"neuroprotection","score":0.8130000233650208},{"id":"https://openalex.org/keywords/grp78","display_name":"GRP78","score":0.824999988079071},{"id":"https://openalex.org/keywords/astrocyte-activation","display_name":"astrocyte activation","score":0.7919999957084656},{"id":"https://openalex.org/keywords/motor-impairment","display_name":"motor impairment","score":0.8949999809265137},{"id":"https://openalex.org/keywords/drug-target-engagement","display_name":"drug-target engagement","score":0.47699999809265137},{"id":"https://openalex.org/keywords/combination-therapy","display_name":"combination therapy","score":0.5690000057220459}],"concepts":[{"id":"https://openalex.org/C2777172819","wikidata":"https://www.wikidata.org/wiki/Q240642","display_name":"Valproic Acid","level":3,"score":0.8260403871536255},{"id":"https://openalex.org/C25498285","wikidata":"https://www.wikidata.org/wiki/Q1981368","display_name":"Neuroprotection","level":2,"score":0.6876654028892517},{"id":"https://openalex.org/C2779965356","wikidata":"https://www.wikidata.org/wiki/Q4811564","display_name":"Astrogliosis","level":3,"score":0.6767515540122986},{"id":"https://openalex.org/C169760540","wikidata":"https://www.wikidata.org/wiki/Q207011","display_name":"Neuroscience","level":1,"score":0.485056072473526},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.46316230297088623},{"id":"https://openalex.org/C98274493","wikidata":"https://www.wikidata.org/wiki/Q128406","display_name":"Pharmacology","level":1,"score":0.45992738008499146},{"id":"https://openalex.org/C2777981454","wikidata":"https://www.wikidata.org/wiki/Q7370237","display_name":"Rotarod performance test","level":3,"score":0.43764299154281616},{"id":"https://openalex.org/C2776925932","wikidata":"https://www.wikidata.org/wiki/Q1755122","display_name":"Neurodegeneration","level":3,"score":0.410605788230896},{"id":"https://openalex.org/C2778186239","wikidata":"https://www.wikidata.org/wiki/Q41571","display_name":"Epilepsy","level":2,"score":0.3883013129234314},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.3865886330604553},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.3503159284591675},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.31726017594337463},{"id":"https://openalex.org/C2985495968","wikidata":"https://www.wikidata.org/wiki/Q422453","display_name":"Motor activity","level":2,"score":0.16587978601455688},{"id":"https://openalex.org/C529278444","wikidata":"https://www.wikidata.org/wiki/Q47273","display_name":"Central nervous system","level":2,"score":0.09990942478179932}],"mesh":[{"descriptor_ui":"D000067699","descriptor_name":"Ataxin-3","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D000067699","descriptor_name":"Ataxin-3","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D000067699","descriptor_name":"Ataxin-3","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D000091342","descriptor_name":"Endoplasmic Reticulum Chaperone BiP","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000091342","descriptor_name":"Endoplasmic Reticulum Chaperone BiP","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000091342","descriptor_name":"Endoplasmic Reticulum Chaperone BiP","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001253","descriptor_name":"Astrocytes","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D001253","descriptor_name":"Astrocytes","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D001253","descriptor_name":"Astrocytes","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006360","descriptor_name":"Heat-Shock Proteins","qualifier_ui":"Q000096","qualifier_name":"biosynthesis","is_major_topic":false},{"descriptor_ui":"D006360","descriptor_name":"Heat-Shock Proteins","qualifier_ui":"Q000096","qualifier_name":"biosynthesis","is_major_topic":false},{"descriptor_ui":"D006360","descriptor_name":"Heat-Shock Proteins","qualifier_ui":"Q000096","qualifier_name":"biosynthesis","is_major_topic":false},{"descriptor_ui":"D006360","descriptor_name":"Heat-Shock Proteins","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D006360","descriptor_name":"Heat-Shock Proteins","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D006360","descriptor_name":"Heat-Shock Proteins","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009154","descriptor_name":"Mutation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009154","descriptor_name":"Mutation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009154","descriptor_name":"Mutation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D014635","descriptor_name":"Valproic Acid","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":false},{"descriptor_ui":"D014635","descriptor_name":"Valproic Acid","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":false},{"descriptor_ui":"D014635","descriptor_name":"Valproic Acid","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D017827","descriptor_name":"Machado-Joseph Disease","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D019680","descriptor_name":"Trinucleotide Repeat Expansion","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D019680","descriptor_name":"Trinucleotide Repeat Expansion","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D019680","descriptor_name":"Trinucleotide Repeat Expansion","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D019680","descriptor_name":"Trinucleotide Repeat Expansion","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D019680","descriptor_name":"Trinucleotide Repeat Expansion","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D019680","descriptor_name":"Trinucleotide Repeat Expansion","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":8,"locations":[{"id":"doi:10.1371/journal.pone.0141610","doi":"https://doi.org/10.1371/journal.pone.0141610","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0141610","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0141610&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLOS ONE","raw_type":"journal-article"},{"id":"pmid:26505994","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/26505994","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PloS one","raw_type":"Journal Article"},{"id":"pmh:oai:europepmc.org:3610738","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/4624233","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"Text"},{"id":"pmh:oai:doaj.org/article:8961ab4612054489a68cfa73e0f680b5","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/8961ab4612054489a68cfa73e0f680b5","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLoS ONE, Vol 10, Iss 10, p e0141610 (2015)","raw_type":"article"},{"id":"pmh:oai:estudogeral.uc.pt:10316/109271","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10316/109271","pdf_url":null,"source":{"id":"https://openalex.org/S4306402070","display_name":"Estudo Geral (Universidad de Coimbra)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I76903346","host_organization_name":"University of Coimbra","host_organization_lineage":["https://openalex.org/I76903346"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:figshare.com:article/1586202","doi":null,"is_oa":true,"landing_page_url":"https://figshare.com/articles/dataset/_Limited_Effect_of_Chronic_Valproic_Acid_Treatment_in_a_Mouse_Model_of_Machado_Joseph_Disease_/1586202","pdf_url":null,"source":{"id":"https://openalex.org/S4377196282","display_name":"Figshare","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I4210132348","host_organization_name":"Figshare (United Kingdom)","host_organization_lineage":["https://openalex.org/I4210132348"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"","raw_type":"Dataset"},{"id":"pmh:oai:repositorium.sdum.uminho.pt:1822/51425","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/1822/51425","pdf_url":null,"source":{"id":"https://openalex.org/S4306402433","display_name":"Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"","raw_type":"journal article"},{"id":"pmh:oai:repositorium.uminho.pt:1822/51425","doi":null,"is_oa":true,"landing_page_url":"https://hdl.handle.net/1822/51425","pdf_url":null,"source":{"id":"https://openalex.org/S4306402433","display_name":"Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"journal article"}],"best_oa_location":{"id":"doi:10.1371/journal.pone.0141610","doi":"https://doi.org/10.1371/journal.pone.0141610","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0141610","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0141610&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLOS ONE","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"sustainable_development_goals_aurora":[{"id":"https://metadata.un.org/sdg/3","score":0.8100000023841858,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[{"id":"https://openalex.org/F4320320050","display_name":"Ataxia UK","ror":"https://ror.org/03192vt15"}],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W1885640155.pdf","grobid_xml":"https://content.openalex.org/works/W1885640155.grobid-xml"},"referenced_works_count":65,"referenced_works":["https://openalex.org/W114637156","https://openalex.org/W125678881","https://openalex.org/W1480365384","https://openalex.org/W1577751242","https://openalex.org/W1643390591","https://openalex.org/W1798753198","https://openalex.org/W1964184380","https://openalex.org/W1964372470","https://openalex.org/W1968109664","https://openalex.org/W1969858573","https://openalex.org/W1971334707","https://openalex.org/W1978177900","https://openalex.org/W1988325154","https://openalex.org/W1996277745","https://openalex.org/W1996785364","https://openalex.org/W2001025550","https://openalex.org/W2008675515","https://openalex.org/W2011484579","https://openalex.org/W2011925152","https://openalex.org/W2014739853","https://openalex.org/W2017000564","https://openalex.org/W2018759446","https://openalex.org/W2022486367","https://openalex.org/W2030301626","https://openalex.org/W2033189345","https://openalex.org/W2034924202","https://openalex.org/W2035623537","https://openalex.org/W2041597162","https://openalex.org/W2060155551","https://openalex.org/W2066853749","https://openalex.org/W2067701929","https://openalex.org/W2070028657","https://openalex.org/W2073012734","https://openalex.org/W2076991511","https://openalex.org/W2082629538","https://openalex.org/W2082946376","https://openalex.org/W2086540681","https://openalex.org/W2088077925","https://openalex.org/W2094916309","https://openalex.org/W2096324109","https://openalex.org/W2096949620","https://openalex.org/W2099813190","https://openalex.org/W2104692716","https://openalex.org/W2105021828","https://openalex.org/W2105180380","https://openalex.org/W2107522917","https://openalex.org/W2108162932","https://openalex.org/W2108727717","https://openalex.org/W2112434293","https://openalex.org/W2123588104","https://openalex.org/W2123895134","https://openalex.org/W2124458807","https://openalex.org/W2125209829","https://openalex.org/W2131323825","https://openalex.org/W2140277032","https://openalex.org/W2140741960","https://openalex.org/W2144872567","https://openalex.org/W2151813227","https://openalex.org/W2153218957","https://openalex.org/W2160737910","https://openalex.org/W2165497333","https://openalex.org/W2167190147","https://openalex.org/W2167746292","https://openalex.org/W2357554713","https://openalex.org/W2412159077"],"related_works":["https://openalex.org/W4389611445","https://openalex.org/W1995458780","https://openalex.org/W1504288624","https://openalex.org/W1971213127","https://openalex.org/W2022320406","https://openalex.org/W138744316","https://openalex.org/W2098555823","https://openalex.org/W1544587770","https://openalex.org/W2006645013","https://openalex.org/W4224989634"],"abstract_inverted_index":{"Machado-Joseph":[0],"disease":[1],"(MJD)":[2],"is":[3],"an":[4],"inherited":[5],"neurodegenerative":[6],"disease,":[7],"caused":[8],"by":[9],"a":[10,43,156],"CAG":[11],"repeat":[12],"expansion":[13],"within":[14],"the":[15,32,70,78,90,105,145],"coding":[16],"region":[17],"of":[18,35,61,81,129,132,142,147,150,153],"ATXN3":[19,106],"gene,":[20],"and":[21,49,58,96,101,109],"which":[22],"currently":[23],"lacks":[24],"effective":[25,54],"treatment.":[26],"In":[27,140],"this":[28],"work":[29],"we":[30],"tested":[31],"therapeutic":[33],"efficacy":[34],"chronic":[36,66,117],"treatment":[37,68,118],"with":[38,45,159],"valproic":[39],"acid":[40],"(VPA)":[41],"(200mg/kg),":[42],"compound":[44],"known":[46,134],"neuroprotection":[47],"activity,":[48],"previously":[50],"shown":[51],"to":[52,121],"be":[53,165],"in":[55,69,77,89,111,155],"cell,":[56],"fly":[57],"nematode":[59],"models":[60],"MJD.":[62],"We":[63],"show":[64],"that":[65],"VPA":[67,116,151,154],"CMVMJD135":[71],"mouse":[72],"model":[73],"had":[74],"limited":[75,143],"effects":[76],"motor":[79,91],"deficits":[80],"these":[82],"mice,":[83],"seen":[84],"mostly":[85],"at":[86,126],"late":[87],"stages":[88],"swimming,":[92],"beam":[93],"walk,":[94],"rotarod":[95],"spontaneous":[97],"locomotor":[98],"activity":[99],"tests,":[100],"did":[102],"not":[103],"modify":[104],"inclusion":[107],"load":[108],"astrogliosis":[110],"affected":[112],"brain":[113],"regions.":[114],"However,":[115],"was":[119],"able":[120],"increase":[122],"GRP78":[123],"protein":[124],"levels":[125],"30":[127],"weeks":[128],"age,":[130],"one":[131],"its":[133],"neuroprotective":[135],"effects,":[136],"confirming":[137],"target":[138],"engagement.":[139],"spite":[141],"results,":[144],"use":[146],"another":[148],"dosage":[149],"or":[152],"combined":[157],"therapy":[158],"molecules":[160],"targeting":[161],"other":[162],"pathways,":[163],"cannot":[164],"excluded":[166],"as":[167],"potential":[168],"strategies":[169],"for":[170],"MJD":[171],"therapeutics.":[172]},"counts_by_year":[{"year":2026,"cited_by_count":1},{"year":2025,"cited_by_count":2},{"year":2024,"cited_by_count":1},{"year":2023,"cited_by_count":1},{"year":2022,"cited_by_count":2},{"year":2021,"cited_by_count":2},{"year":2020,"cited_by_count":3},{"year":2019,"cited_by_count":3},{"year":2018,"cited_by_count":7},{"year":2017,"cited_by_count":2},{"year":2016,"cited_by_count":2}],"updated_date":"2026-10-08T09:06:37.294493","created_date":"2025-10-10T00:00:00"}