{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T01:38:52Z","timestamp":1790041132053,"version":"4.0.1"},"reference-count":32,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T00:00:00Z","timestamp":1766361600000},"content-version":"vor","delay-in-days":21,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T00:00:00Z","timestamp":1764547200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100014188","name":"Ministry of Science and ICT, South Korea","doi-asserted-by":"publisher","award":["1711198445"],"award-info":[{"award-number":["1711198445"]}],"id":[{"id":"10.13039\/501100014188","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100014188","name":"Ministry of Science and ICT, South Korea","doi-asserted-by":"publisher","award":["KK2331\u201010"],"award-info":[{"award-number":["KK2331\u201010"]}],"id":[{"id":"10.13039\/501100014188","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100014188","name":"Ministry of Science and ICT, South Korea","doi-asserted-by":"publisher","award":["2710008773"],"award-info":[{"award-number":["2710008773"]}],"id":[{"id":"10.13039\/501100014188","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100014188","name":"Ministry of Science and ICT, South Korea","doi-asserted-by":"publisher","award":["KK\u20102410"],"award-info":[{"award-number":["KK\u20102410"]}],"id":[{"id":"10.13039\/501100014188","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disorder characterized by balance and gait disturbance, muscle coordination deficits, and dysarthria as a primary symptom. SCA1 is caused by the expansion of CAG repeats in the\n                    <jats:italic>ATXN1<\/jats:italic>\n                    gene, leading to a polyglutamine(polyQ) tract in the Ataxin\u20101 protein. In this study, the effects of KDS2010, an inhibitor of monoamine oxidase\u2010B (MAO\u2010B), were evaluated in a transgenic mouse model of SCA1 (SCA1\n                    <jats:sup>154Q\/2Q<\/jats:sup>\n                    ) disease model using the rotarod, hindlimb, and open field tests. In both males and females, oral administration of KDS2010 significantly improved latency in the rotarod test and the hindlimb clasping phenotype without changing muscle weight. In the open field test, a notable improvement in moving distance was observed particularly in females. Treatment with KDS2010 slowed molecular layer atrophy and restored the reduced number of Purkinje cells in the cerebellum of SCA1 model mice, accompanied by decreased levels of glial fibrillary acidic protein (GFAP) and reduced expression of monoamine oxidase\u2010B (MAO\u2010B). These results indicate that KDS2010 ameliorated the behavioral pathology of SCA1 by attenuating GFAP upregulation and preventing Purkinje cell loss. Our findings demonstrate that KDS2010 improves SCA1\u2010related behavioral deficits by reducing GFAP expression, preserving Purkinje cell numbers, and decreasing cerebellar MAO\u2010B levels\u2014molecular markers of SCA1, without affecting muscle size.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                      <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc70302-gra-0001-m.png\">\n                        <jats:alt-text>image<\/jats:alt-text>\n                      <\/jats:graphic>\n                    <\/jats:boxed-text>\n                  <\/jats:p>","DOI":"10.1111\/jnc.70302","type":"journal-article","created":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T13:51:32Z","timestamp":1766411492000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Oral\n                    <scp>KDS2010<\/scp>\n                    , a Monoamine Oxidase\u2010B (\n                    <scp>MAO<\/scp>\n                    \u2010B) Inhibitor, Slows the Deterioration of Motor Coordination in Genetic\n                    <scp>SCA1<\/scp>\n                    Models by Inhibiting Astrocytic MAO\u2010B\u2010Mediated Inflammation"],"prefix":"10.1111","volume":"169","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3366-3831","authenticated-orcid":false,"given":"Dong Ho","family":"Woo","sequence":"first","affiliation":[{"name":"Center for Global Biopharmaceutical Research Korea Institute of Toxicology  Daejeon South Korea"},{"name":"Human and Environmental Toxicology University of Science and Technology  Daejeon Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ju\u2010Hyun","family":"Jeon","sequence":"additional","affiliation":[{"name":"Center for the Rare Disease Therapeutic Technology Korea Research Institute of Chemical Technology  Daejeon South Korea"},{"name":"Graduate School of New Drug Discovery and Development Chungnam National University  Daejeon South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hye\u2010Jin","family":"Ryu","sequence":"additional","affiliation":[{"name":"Center for the Rare Disease Therapeutic Technology Korea Research Institute of Chemical Technology  Daejeon South Korea"},{"name":"Graduate School of New Drug Discovery and Development Chungnam National University  Daejeon South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gyeong Min","family":"Lee","sequence":"additional","affiliation":[{"name":"Center for the Rare Disease Therapeutic Technology Korea Research Institute of Chemical Technology  Daejeon South Korea"},{"name":"Graduate School of New Drug Discovery and Development Chungnam National University  Daejeon South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hye\u2010Won","family":"Lim","sequence":"additional","affiliation":[{"name":"Center for Global Biopharmaceutical Research Korea Institute of Toxicology  Daejeon South Korea"},{"name":"Human and Environmental Toxicology University of Science and Technology  Daejeon Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ki Duk","family":"Park","sequence":"additional","affiliation":[{"name":"Brain Science Institute Korea Institute of Science and Technology (KIST)  Seoul South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kwang Lae","family":"Hoe","sequence":"additional","affiliation":[{"name":"Graduate School of New Drug Discovery and Development Chungnam National University  Daejeon South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang Dal","family":"Rhee","sequence":"additional","affiliation":[{"name":"Center for the Rare Disease Therapeutic Technology Korea Research Institute of Chemical Technology  Daejeon South Korea"},{"name":"Graduate School of New Drug Discovery and Development Chungnam National University  Daejeon South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,12,22]]},"reference":[{"key":"e_1_2_11_2_1","article-title":"Abnormalities in the Climbing Fiber\u2013Purkinje Cell Circuit Associated With Early\u2010Onset Motor Deficits in SCA1 Mice","volume":"11","author":"Barnes J. 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