{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T08:46:58Z","timestamp":1778834818401,"version":"3.51.4"},"reference-count":22,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2014,5,28]],"date-time":"2014-05-28T00:00:00Z","timestamp":1401235200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Spinocerebellar ataxia type 2 (<jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>2) is a hereditary neurodegenerative disorder caused by the expansion of the trinucleotide <jats:styled-content style=\"fixed-case\">CAG<\/jats:styled-content> repeats encoding elongated polyglutamine tract in ataxin\u20102, the <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>2 gene product. Polyglutamine diseases comprise nine genetic entities, including seven different forms of spinocerebellar ataxias, <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>untington's disease, and spinal and bulbar muscular atrophy. These are pathologically characterized by neuronal loss and intranuclear aggregates or inclusions of mutant proteins including expanded polyglutamine in selected neuronal groups. Previously, we examined immunolocalization of ubiquitin, expanded polyglutamine (probed by 1<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>2 antibody), and ataxin\u20102 in genetically confirmed <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>2 patients. In the present study, we expanded this approach by distinguishing different patterns of subcellular 1<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>2 immunoreactivity (\u201cgranular cytoplasmic,\u201d \u201ccytoplasmic and nuclear\u201d and \u201cnuclear with inclusions.\u201d) and by quantifying their regional frequencies in three autopsied <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>2 brains at different stage of the disease. Comparison with neuronal loss and gliosis revealed that overall 1<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>2 immunoreactivity was paralleled with their severity. Furthermore, appearance of granular cytoplasmic pattern corresponded to early stage, cytoplasmic and nuclear pattern to active stage, and nuclear with inclusions pattern to final stage. We conclude that this 1<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>2\u2010immunoreactive typing may be useful for evaluating the overall severity and extent of affected regions and estimating the neuropathological stage of <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>2.<\/jats:p>","DOI":"10.1111\/bpa.12146","type":"journal-article","created":{"date-parts":[[2014,3,27]],"date-time":"2014-03-27T03:42:53Z","timestamp":1395891773000},"page":"599-606","source":"Crossref","is-referenced-by-count":19,"title":["Neuropathological Staging of Spinocerebellar Ataxia Type 2 by Semiquantitative 1<scp>C<\/scp>2\u2010Positive Neuron Typing. Nuclear Translocation of Cytoplasmic 1<scp>C<\/scp>2 Underlies Disease Progression of Spinocerebellar Ataxia Type 2"],"prefix":"10.1111","volume":"24","author":[{"given":"Shigeru","family":"Koyano","sequence":"first","affiliation":[{"name":"Department of Neurology and Stroke Medicine Yokohama City University Graduate School of Medicine  Yokohama Kanagawa Japan"},{"name":"Laboratory of Structural Neuropathology Tokyo Metropolitan Institute of Medical Science  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saburo","family":"Yagishita","sequence":"additional","affiliation":[{"name":"Department of Pathology Kanagawa Rehabilitation Center  Atsugi Kanagawa Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiyuki","family":"Kuroiwa","sequence":"additional","affiliation":[{"name":"Department of Neurology Teikyo University School of Medicine University Hospital Mizonokuchi  Kawasaki Kanagawa Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fumiaki","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Department of Neurology and Stroke Medicine Yokohama City University Graduate School of Medicine  Yokohama Kanagawa Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiki","family":"Uchihara","sequence":"additional","affiliation":[{"name":"Laboratory of Structural Neuropathology Tokyo Metropolitan Institute of Medical Science  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,5,28]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2005.02.063"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature02998"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/118.6.1573"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1006\/exnr.2000.7479"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-005-1014-8"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/dds427"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12264-010-0113-2"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/79162"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2010.01176.x"},{"key":"e_1_2_6_11_1","first-page":"255","article-title":"Autosomal dominant spinocerebellar degenerations. Clinical, pathological, and genetic correlations","volume":"155","author":"Iwabuchi K","year":"1999","journal-title":"Rev Neurol (Paris)"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.73.4.450"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(99)00656-4"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-008-0019-y"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1159\/000007974"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(89)90159-7"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf060"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80943-5"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh188"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awg234"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1080\/00207450600936668"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-011-0265-2"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1325.040"}],"container-title":["Brain Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fbpa.12146","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bpa.12146","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T02:53:50Z","timestamp":1696301630000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bpa.12146"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,5,28]]},"references-count":22,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2014,11]]}},"alternative-id":["10.1111\/bpa.12146"],"URL":"https:\/\/doi.org\/10.1111\/bpa.12146","archive":["Portico"],"relation":{},"ISSN":["1015-6305","1750-3639"],"issn-type":[{"value":"1015-6305","type":"print"},{"value":"1750-3639","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,5,28]]}}}