{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,11]],"date-time":"2026-11-11T21:37:24Z","timestamp":1794433044723,"version":"4.9.1"},"reference-count":38,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T00:00:00Z","timestamp":1667433600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Sapienza University of Rome","award":["2018\/2019"],"award-info":[{"award-number":["2018\/2019"]}]},{"name":"Sapienza University of Rome","award":["0942"],"award-info":[{"award-number":["0942"]}]},{"name":"Sapienza University of Rome","award":["FOE D.M. 865\/2019"],"award-info":[{"award-number":["FOE D.M. 865\/2019"]}]},{"name":"EHDN seed fund","award":["2018\/2019"],"award-info":[{"award-number":["2018\/2019"]}]},{"name":"EHDN seed fund","award":["0942"],"award-info":[{"award-number":["0942"]}]},{"name":"EHDN seed fund","award":["FOE D.M. 865\/2019"],"award-info":[{"award-number":["FOE D.M. 865\/2019"]}]},{"name":"AICH","award":["2018\/2019"],"award-info":[{"award-number":["2018\/2019"]}]},{"name":"AICH","award":["0942"],"award-info":[{"award-number":["0942"]}]},{"name":"AICH","award":["FOE D.M. 865\/2019"],"award-info":[{"award-number":["FOE D.M. 865\/2019"]}]},{"name":"Fondo Ordinario Enti","award":["2018\/2019"],"award-info":[{"award-number":["2018\/2019"]}]},{"name":"Fondo Ordinario Enti","award":["0942"],"award-info":[{"award-number":["0942"]}]},{"name":"Fondo Ordinario Enti","award":["FOE D.M. 865\/2019"],"award-info":[{"award-number":["FOE D.M. 865\/2019"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The identification of biomarkers for neurodegenerative disorders such as Huntington\u2019s disease (HD) is crucial for monitoring disease progression and therapeutic trial outcomes, especially in the pre-manifest disease stage (pre-HD). In a previous study, we observed that leukocyte telomere length (LTL) was strongly correlated with the estimated time to clinical onset in pre-HD subjects. To validate this hypothesis, we designed a follow-up study in which we analyzed LTL in 45 pre-HD stage subjects at baseline (T0) and then again after clinical onset at follow-up (T1); the follow-up interval was about 3 years, and the CAG range was 39\u201351 repeats; 90 peripheral blood mononuclear cell samples (PBMCs) were obtained from the Enroll-HD biorepository. In pre-HD subjects at T0, LTL was significantly reduced by 22% compared to the controls and by 14% from T0 at T1. No relationship was observed between the LTL and CAG numbers in subjects carrying different CAG repeats at T0 and at T1, suggesting that LTL reduction occurs independently of CAG number in pre-HD subjects. ROC curve analysis was used to test the validity of LTL as a potential biomarker of HD progression and showed that LTL measurement is extremely accurate in discriminating pre-HD subjects from the controls and even pre-HD from manifest HD, thus yielding a robust prognostic value in pre-HD subjects.<\/jats:p>","DOI":"10.3390\/ijms232113449","type":"journal-article","created":{"date-parts":[[2022,11,4]],"date-time":"2022-11-04T03:28:10Z","timestamp":1667532490000},"page":"13449","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Leukocyte Telomere Length as Potential Biomarker of HD Progression: A Follow-Up Study"],"prefix":"10.3390","volume":"23","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"}]},{"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"}]},{"given":"Ivan","family":"Arisi","sequence":"additional","affiliation":[{"name":"Institute of Translational Pharmacology, National Research Council, 00133 Rome, Italy"},{"name":"Fondazione European Brain Research Institute (EBRI) Rita Levi-Montalcini, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessia","family":"Fiore","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnology, La Sapienza University, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8671-7958","authenticated-orcid":false,"given":"Marina","family":"Frontali","sequence":"additional","affiliation":[{"name":"Institute of Translational Pharmacology, National Research Council, 00133 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa Maria","family":"Corbo","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnology, La Sapienza University, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1136\/jmg.2009.066399","article-title":"Wild-Type but Not Mutant Huntingtin Modulates the Transcriptional Activity of Liver X Receptors","volume":"46","author":"Futter","year":"2009","journal-title":"J. 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