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Immunohistochemical studies using the 1C2 antibody for polyglutamine expansion have detected characteristic intranuclear inclusions (INIs) in affected neurons in HD. Further, <jats:italic>in vitro<\/jats:italic> and mouse models of HD have shown that the INIs recruit several proteins relating to RNA splicing and translation. In the present study, we immunohistochemically investigated the association of INIs with various heterogeneous nuclear ribonucleoproteins in the cerebral cortex of four autopsy cases of HD. Fused in sarcoma (FUS) was colocalized with 1C2\u2010positive nuclear inclusions in all examined cases. Localization of poly (rC)\u2010binding protein 1 (PCBP1) in 1C2\u2010positive nuclear inclusions was also observed. Double immunofluorescence revealed complete or partial loss of the normal, diffuse nuclear distribution of FUS or PCBP1 in neurons with 1C2\u2010positive nuclear inclusions. This maldistribution of FUS in cortical neurons suggests a severe disturbance of messenger RNA processing, which may be a common pathogenetic mechanism of FUS\u2010related familial amyotrophic lateral sclerosis.<\/jats:p>","DOI":"10.1111\/neup.12600","type":"journal-article","created":{"date-parts":[[2019,10,10]],"date-time":"2019-10-10T00:39:14Z","timestamp":1570667954000},"page":"358-367","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Expanded polyglutamine impairs normal nuclear distribution of fused in sarcoma and poly (rC)\u2010binding protein 1 in Huntington's disease"],"prefix":"10.1111","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5342-0648","authenticated-orcid":false,"given":"Shinichiro","family":"Mori","sequence":"first","affiliation":[{"name":"Department of Neuropathology Graduate School of Medical Sciences, Kyushu University  Fukuoka Japan"},{"name":"Department of Neurology, 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