{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T04:21:58Z","timestamp":1768278118482,"version":"3.49.0"},"reference-count":52,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,5,3]],"date-time":"2017-05-03T00:00:00Z","timestamp":1493769600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100005614","name":"Biogen","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005614","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000890","name":"National Multiple Sclerosis Society","doi-asserted-by":"publisher","award":["RG4078"],"award-info":[{"award-number":["RG4078"]}],"id":[{"id":"10.13039\/100000890","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000890","name":"National Multiple Sclerosis Society","doi-asserted-by":"publisher","award":["RG4639"],"award-info":[{"award-number":["RG4639"]}],"id":[{"id":"10.13039\/100000890","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:label\/><jats:p>Multiple sclerosis (<jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content>) is considered a primary autoimmune disease; however, this view is increasingly being challenged in basic and clinical science arenas because of the growing body of clinical trials\u2019 data showing that exclusion of immune cells from the <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content> only modestly slows disease progression to disability. Accordingly, there is significant need for expanding the scope of potential disease mechanisms to understand the etiology of <jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content>. Concomitantly, the use of a broader range of pre\u2010clinical animal models for characterizing existing efficacious clinical treatments may elucidate additional or unexpected mechanisms of action for these drugs that augment insight into <jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content> etiology. Herein, we explore the <jats:italic>in\u00a0vivo<\/jats:italic> mechanism of action of dimethyl fumarate, which has been shown to suppress oxidative stress and immune cell responses in psoriasis and <jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content>. Rather than studying this compound in the context of an experimental autoimmune\u2010induced attack on the <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>, we have used a genetic model of hypomyelination, male <jats:italic>rumpshaker<\/jats:italic> (<jats:italic>rsh<\/jats:italic>) mice, which exhibit oligodendrocyte metabolic stress and startle\u2010induced subcortical myoclonus during development and into adulthood. We find that myoclonus is reduced 30\u201050% in treated mutants but we do not detect substantial changes in metabolic or oxidative stress response pathways, cytokine modulation, or myelin thickness (assessed by <jats:sc>anova<\/jats:sc>). All procedures involving vertebrate animals in this study were reviewed and approved by the <jats:styled-content style=\"fixed-case\">IACUC<\/jats:styled-content> committee at Wayne State University.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>\n<jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc14035-fig-0007-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text>\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/jnc.14035","type":"journal-article","created":{"date-parts":[[2017,4,6]],"date-time":"2017-04-06T07:54:44Z","timestamp":1491465284000},"page":"103-117","source":"Crossref","is-referenced-by-count":8,"title":["Dimethyl fumarate ameliorates myoclonus stemming from protein misfolding in oligodendrocytes"],"prefix":"10.1111","volume":"142","author":[{"given":"Cherie M.","family":"Southwood","sequence":"first","affiliation":[{"name":"Center for Molecular Medicine and Genetics Wayne State University School of Medicine Detroit MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danielle M.","family":"Garshott","sequence":"additional","affiliation":[{"name":"Carman and Ann Adams Department of Pediatrics Wayne State University School of Medicine Detroit MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chelsea R.","family":"Richardson","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Genetics Wayne State University School of Medicine Detroit MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Navid","family":"Seraji\u2010Bozorgzad","sequence":"additional","affiliation":[{"name":"Department of Neurology Wayne State University School of Medicine Detroit MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew M.","family":"Fribley","sequence":"additional","affiliation":[{"name":"Carman and Ann Adams Department of Pediatrics Wayne State University School of Medicine Detroit MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7446-8990","authenticated-orcid":false,"given":"Alexander","family":"Gow","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Genetics Wayne State University School of Medicine Detroit MI USA"},{"name":"Carman and Ann Adams Department of Pediatrics Wayne State University School of Medicine Detroit MI USA"},{"name":"Department of Neurology Wayne State University School of Medicine Detroit MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,5,3]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.10561"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.20842"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms141021021"},{"key":"e_1_2_8_5_1","first-page":"CD010497","article-title":"Oral fumaric acid esters for psoriasis","volume":"8","author":"Atwan A.","year":"2015","journal-title":"Cochrane Database Syst. Rev."},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.22379"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(80)91215-9"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1159\/000048691"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ncl.2009.04.002"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneumeth.2003.10.009"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200808034"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.440050302"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.440090208"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1080\/09674845.2013.11669939"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0166-2236(03)00197-8"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1185\/03007995.2013.849236"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01189804"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1615\/CritRevImmunol.2013007247"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1385\/NMM:4:1-2:73"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.490370504"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.140.4.925"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81553-6"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01217305"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12974-016-0559-x"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1177\/08830738030180090801"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2015.07.147"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1177\/1756285610395653"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.exppara.2010.02.002"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1462-5822.2007.00901.x"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200502086"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awq386"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.13456"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2007.1782"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1610-0387.2012.07996_suppl.x"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2011.01.038"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.490330204"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2015.361"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.5772\/54461"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2006.03094.x"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1603907113"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/875403"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2007.04541.x"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2007.04646.x"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(82)91143-X"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrn1007"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1002\/jemt.1054"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(02)01045-0"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1095\/biolreprod.112.099879"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.3390\/brainsci3041417"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1118-15.2016"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(83)91371-9"},{"key":"e_1_2_8_52_1","first-page":"CD011076","article-title":"Dimethyl fumarate for multiple sclerosis","volume":"4","author":"Xu Z.","year":"2015","journal-title":"Cochrane Database Syst. Rev."},{"key":"e_1_2_8_53_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI60363"}],"container-title":["Journal of Neurochemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjnc.14035","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jnc.14035","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T19:27:37Z","timestamp":1696274857000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jnc.14035"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,5,3]]},"references-count":52,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2017,7]]}},"alternative-id":["10.1111\/jnc.14035"],"URL":"https:\/\/doi.org\/10.1111\/jnc.14035","archive":["Portico"],"relation":{},"ISSN":["0022-3042","1471-4159"],"issn-type":[{"value":"0022-3042","type":"print"},{"value":"1471-4159","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,5,3]]}}}