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In human cell culture, expression of mutant Huntingtin carrying 80–97 polyglutamine repeats drives aggregation and phosphorylation of endogenous TDP-43, while non-pathogenic Huntingtin with 25 repeats remains ineffective. Mutant Huntingtin aggregation occurs first, followed by pTDP-43 accumulation and co-localization at mHtt inclusions, mirroring observations in Huntington patient brains. Detergent-insoluble fractions containing mHtt–pTDP-43 co-aggregates act as seeds that promote further TDP-43 aggregation in cells. The prion protein PrPC reduces mutant Huntingtin aggregation in parallel with decreased TDP-43 aggregation and phosphorylation, reinforcing a tight mechanistic link between the two proteins.",{"@graph":69,"@context":105},[70,84],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":40,"@type":76,"position":81},"https://docshare.wps.com/document/research-report/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/phosphorylated-and-aggregated-tdp-43-with-seeding-properties-induced-upon-mutant-huntingtin-polyglutamine-expression-in-human-cellular-models/24323/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/phosphorylated-and-aggregated-tdp-43-with-seeding-properties-induced-upon-mutant-huntingtin-polyglutamine-expression-in-human-cellular-models/24323.png","ImageObject",300,407,{"name":92,"@type":93},"Logic","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-09-17","2026-05-07",true,{"@type":102,"interactionType":103,"userInteractionCount":8},"InteractionCounter",{"@type":104},"ViewAction","https://schema.org",{"og:url":107,"og:type":108,"og:title":65,"og:site_name":95,"og:description":67},"https://docshare.wps.com/document/phosphorylated-and-aggregated-tdp-43-with-seeding-properties-induced-upon-mutant-huntingtin-polyglutamine-expression-in-human-cellular-models/24323","article",{"robots":110,"canonical":107},"index,follow",{"doc_id":112,"site_id":62},24323,1778188551,{"code":4,"msg":5,"data":115},{"doc_id":112,"user_id":116,"nickname":92,"user_avatar":117,"doc_module":4,"category_id":39,"category_name":40,"doc_title":65,"doc_description":67,"doc_content":66,"file_id":118,"file_url":119,"file_type":120,"file_size":121,"view_count":8,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":122,"language":123,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":66,"faqs":66,"seo_title":124,"seo_description":67,"update_tm":113,"read_time":125},1099513958762,"https://ap-avatar.wpscdn.com/avatar/1000023916a998db790?x-image-process=image/resize,m_fixed,w_180,h_180&k=1784791008015729253","cbCaibmdvZxd0k15","https://ap.wps.com/l/cbCaibmdvZxd0k15","pdf",2634947,18,"English","Phosphorylated and aggregated TDP-43 with seeding properties induced upon mutant Huntingtin polyglutamine expression in human cellular models | PDF",45]