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We analyzed SAFB1 expression in the post\u2010mortem brain tissue of spinocerebellar ataxia (SCA), Huntington\u2019s disease (HD), Multiple sclerosis (MS), Parkinson\u2019s disease patients and controls. In SCA cases, the expression of SAFB1 in the nucleus was increased and there was abnormal and extensive expression in the cytoplasm where it co\u2010localized with the markers of Purkinje cell injury. Significantly, no SAFB1 expression was found in the cerebellar neurons of the dentate nucleus in control or MS patients; however, in SCA patients, SAFB1 expression was increased significantly in both the nucleus and cytoplasm of dentate neurons. In HD, we found that SAFB1 expression was increased in the nucleus and cytoplasm of striatal neurons; however, there was no SAFB1 staining in the striatal neurons of controls. In PD substantia nigra, we did not see any changes in neuronal SAFB1 expression. iCLIP analysis found that SAFB1 crosslink sites within ATXN1 RNA were adjacent to the start and within the glutamine repeat sequence. Further investigation found increased binding of SAFB1 to pathogenic ATXN1\u201085Q mRNA. These novel data strongly suggest SAFB1 contributes to the etiology of SCA and Huntington\u2019s chorea and that it may be a pathological marker of polyglutamine repeat expansion diseases.<\/jats:p>","DOI":"10.1111\/bpa.12872","type":"journal-article","created":{"date-parts":[[2020,6,24]],"date-time":"2020-06-24T21:06:35Z","timestamp":1593032795000},"page":"1041-1055","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Abnormal scaffold attachment factor 1 expression and localization in spinocerebellar ataxias and Huntington\u2019s chorea"],"prefix":"10.1111","volume":"30","author":[{"given":"Nicola","family":"Buckner","sequence":"first","affiliation":[{"name":"Bristol Medical School Translational Health Sciences University of Bristol  Bristol UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin C.","family":"Kemp","sequence":"additional","affiliation":[{"name":"Bristol Medical School Translational 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