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We investigated the relationship between leukocyte telomere length (LTL) and the phenotype of SCA1, SCA2, and SCA3, comparing them with HD. The results showed that LTL was significantly reduced in SCA1 and SCA3 patients, while LTL was significantly longer in SCA2 patients. A significant negative relationship between LTL and age was observed in SCA1 but not in SCA2 subjects. LTL of SCA3 patients depend on both patient\u2019s age and disease duration. The number of CAG repeats did not affect LTL in the three SCAs. Since LTL is considered an indirect marker of an inflammatory response and oxidative damage, our data suggest that in SCA1 inflammation is present already at an early stage of disease similar to in HD, while in SCA3 inflammation and impaired antioxidative processes are associated with disease progression. Interestingly, in SCA2, contrary to SCA1 and SCA3, the length of leukocyte telomeres does not reduce with age. We have observed that SCAs and HD show a differing behavior in LTL for each subtype, which could constitute relevant biomarkers if confirmed in larger cohorts and longitudinal studies.<\/jats:p>","DOI":"10.3390\/antiox11081436","type":"journal-article","created":{"date-parts":[[2022,7,24]],"date-time":"2022-07-24T22:49:02Z","timestamp":1658702942000},"page":"1436","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Leukocyte Telomere Length Variability as a Potential Biomarker in Patients with PolyQ Diseases"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5565-6379","authenticated-orcid":false,"given":"Daniela","family":"Scarabino","sequence":"first","affiliation":[{"name":"Institute of Molecular Biology and Pathology, National Research Council, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9921-8450","authenticated-orcid":false,"given":"Liana","family":"Veneziano","sequence":"additional","affiliation":[{"name":"Institute of Translational Pharmacology, National Research Council, 00133 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessia","family":"Fiore","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnology, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4272-9292","authenticated-orcid":false,"given":"Suran","family":"Nethisinghe","sequence":"additional","affiliation":[{"name":"Ataxia Center, Department of Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, University College, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1656-5543","authenticated-orcid":false,"given":"Elide","family":"Mantuano","sequence":"additional","affiliation":[{"name":"Institute of Translational Pharmacology, National Research Council, 00133 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0697-3271","authenticated-orcid":false,"given":"Hector","family":"Garcia-Moreno","sequence":"additional","affiliation":[{"name":"Ataxia Center, Department of Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, University College, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2635-1699","authenticated-orcid":false,"given":"Gianmarco","family":"Bellucci","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Mental Health and Sensory Organs, Centre for Experimental Neurological Therapies (CENTERS), Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nita","family":"Solanky","sequence":"additional","affiliation":[{"name":"Ataxia Center, Department of Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, University College, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Morello","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine and Surgery, Tor Vergata University, 00133 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6367-1843","authenticated-orcid":false,"given":"Ginevra","family":"Zanni","sequence":"additional","affiliation":[{"name":"Unit of Neuromuscolar and Neurodegenerative Disorders, Department of Neurosciences, Bambino Ges\u00f9 Children\u2019s Research Hospital, IRCCS, 00100 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1091-2498","authenticated-orcid":false,"given":"Rosa Maria","family":"Corbo","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnology, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Giunti","sequence":"additional","affiliation":[{"name":"Ataxia Center, Department of Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, University College, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"707","DOI":"10.3389\/fnins.2020.00707","article-title":"Aberrant Cerebellar Circuitry in the Spinocerebellar Ataxias","volume":"14","author":"Robinson","year":"2020","journal-title":"Front. 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