{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T02:17:36Z","timestamp":1791253056908,"version":"4.1.0"},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2013,7,29]],"date-time":"2013-07-29T00:00:00Z","timestamp":1375056000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2014,2]]},"abstract":"<jats:p>A nuclear protein, transactivation response (<jats:styled-content style=\"fixed-case\">TAR<\/jats:styled-content>) <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> binding protein 43\u2009<jats:styled-content style=\"fixed-case\">kDa<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043), is the major component of neuronal cytoplasmic inclusions (<jats:styled-content style=\"fixed-case\">NCIs<\/jats:styled-content>) in frontotemporal lobar degeneration with ubiquitin inclusions (<jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>) and sporadic amyotrophic lateral sclerosis (<jats:styled-content style=\"fixed-case\">SALS<\/jats:styled-content>). While initially thought to be relatively specific to <jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">U<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content>, <jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043 pathology has now been detected in a number of other neurodegenerative diseases, including <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>lzheimer's disease and <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>arkinson's disease. In such tauopathies and \u03b1\u2010synucleinopathies, occurrence of <jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043\u2010positive neuronal cytoplasmic inclusions may be associated with other distinct molecular pathologic processes primarily involving their own pathological proteins, tau and \u03b1\u2013synuclein, respectively (secondary <jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043 proteinopathies). On the other hand, in several polyglutamine (<jats:styled-content style=\"fixed-case\">polyQ<\/jats:styled-content>) diseases, <jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043 appears to play an important pathomechanistic role. Interestingly, intermediate\u2010length <jats:styled-content style=\"fixed-case\">polyQ<\/jats:styled-content> expansions (27\u201333 <jats:styled-content style=\"fixed-case\">Qs<\/jats:styled-content>) in ataxin 2, the causative gene of spinocerebellar ataxia type 2, have recently been reported to be a genetic risk factor for <jats:styled-content style=\"fixed-case\">SALS<\/jats:styled-content>. Here, with a review of the literature, we discuss the relationship between <jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">polyQ<\/jats:styled-content> diseases from the viewpoint of <jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043 neuropathology.<\/jats:p>","DOI":"10.1111\/neup.12053","type":"journal-article","created":{"date-parts":[[2013,7,29]],"date-time":"2013-07-29T10:36:11Z","timestamp":1375094171000},"page":"77-82","source":"Crossref","is-referenced-by-count":35,"title":["<scp>TDP<\/scp>\u201043 pathology in polyglutamine diseases: With reference to amyotrphic lateral sclerosis"],"prefix":"10.1111","volume":"34","author":[{"given":"Yasuko","family":"Toyoshima","sequence":"first","affiliation":[{"name":"Department of Pathology Brain Research Institute University of Niigata  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hitoshi","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Pathology Brain Research Institute University of Niigata  Niigata 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