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Further, polypeptides corresponding to subregions of the PRD sufficed to elicit the toxic effect on fast axonal transport, which was mediated by c-Jun N-terminal kinases (JNKs) and involved PRD binding to one or more SH3-domain containing proteins. Collectively, these data suggested a mechanism whereby polyQ tract expansion in mHTT promotes aberrant PRD exposure and interactions of this domain with SH3 domain-containing proteins including some involved in activation of JNKs. In support, biochemical and immunohistochemical experiments linked aberrant PRD exposure to increased JNK activation in striatal tissues of the zQ175 mouse model and from post-mortem Huntington\u2019s disease patients.<\/jats:p>\n               <jats:p>Together, these findings support a critical role of PRD on mHTT toxicity, suggesting a novel framework for the potential development of therapies aimed to halt or reduce axonal pathology in Huntington\u2019s disease.<\/jats:p>","DOI":"10.1093\/brain\/awad280","type":"journal-article","created":{"date-parts":[[2023,8,26]],"date-time":"2023-08-26T18:30:35Z","timestamp":1693074635000},"page":"2098-2113","source":"Crossref","is-referenced-by-count":15,"title":["Toxic effects of mutant huntingtin in axons are mediated by its proline-rich domain"],"prefix":"10.1093","volume":"147","author":[{"given":"Scott T","family":"Brady","sequence":"first","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]},{"name":"Marine Biological Laboratory , Woods Hole, MA 02543 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nichole A","family":"Mesnard-Hoaglin","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Mays","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]},{"name":"Marine Biological Laboratory , Woods Hole, MA 02543 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes","family":"Priego","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Dziechciowska","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Morris","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]},{"name":"Marine Biological Laboratory , Woods Hole, MA 02543 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minsu","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]},{"name":"Marine Biological Laboratory , Woods Hole, MA 02543 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming Ying","family":"Tsai","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology, University of Illinois at Chicago , Chicago, IL 60612 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer L","family":"Purks","sequence":"additional","affiliation":[{"name":"Marine Biological Laboratory , Woods Hole, MA 02543 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison","family":"Klein","sequence":"additional","affiliation":[{"name":"Marine Biological Laboratory , Woods Hole, MA 02543 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelica","family":"Gaona","sequence":"additional","affiliation":[{"name":"Department of Neurology, Massachusetts General Hospital , Charlestown, MA 02129 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