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This study seeks to investigate whether the number of CAG repeats of <jats:italic>HTT<\/jats:italic> is associated with the risk of developing certain tauopathies and its influence as a modulator of the clinical and neuropathological phenotype. Additionally, it aims to evaluate the potential of polyglutamine staining as a neuropathological screening. We genotyped the <jats:italic>HTT<\/jats:italic> gene CAG repeat number and APOE\u2010\u2130 isoforms in a cohort of patients with neuropathological diagnoses of tauopathies (<jats:italic>n<\/jats:italic>=588), including 34 corticobasal degeneration (CBD), 98 progressive supranuclear palsy (PSP) and 456 Alzheimer's disease (AD). Furthermore, we genotyped a control group of 1070 patients, of whom 44 were neuropathologic controls. We identified significant differences in the number of patients with pathological <jats:italic>HTT<\/jats:italic> expansions in the CBD group (2.7%) and PSP group (3.2%) compared to control subjects (0.2%). A significant increase in the size of the <jats:italic>HTT<\/jats:italic> CAG repeats was found in the AD compared to the control group, influenced by the presence of the Apoliprotein E (APOE)\u2010\u21304 isoform. Post\u2010mortem assessments uncovered tauopathy pathology with positive polyglutamine aggregates, with a slight predominance in the neostriatum for PSP and CBD cases and somewhat greater limbic involvement in the AD case. Our results indicated a link between <jats:italic>HTT<\/jats:italic> CAG repeat expansion with other non\u2010HD pathology, suggesting they could share common neurodegenerative pathways. These findings support that genetic or histological screening for <jats:italic>HTT<\/jats:italic> repeat expansions should be considered in tauopathies.<\/jats:p>","DOI":"10.1111\/bpa.13250","type":"journal-article","created":{"date-parts":[[2024,2,29]],"date-time":"2024-02-29T02:00:03Z","timestamp":1709172003000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Huntingtin <scp>CAG<\/scp> repeats in neuropathologically confirmed tauopathies: Novel insights"],"prefix":"10.1111","volume":"34","author":[{"given":"Sergio","family":"P\u00e9rez\u2010Oliveira","sequence":"first","affiliation":[{"name":"Laboratory of Genetics Hospital Universitario Central de Asturias  Oviedo Spain"},{"name":"Instituto de Investigaci\u00f3n Sanitaria del Principado de Asturias (ISPA)  Oviedo 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