{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:02:06Z","timestamp":1791302526376,"version":"4.1.0"},"reference-count":31,"publisher":"Wiley","issue":"14","license":[{"start":{"date-parts":[[2012,9,24]],"date-time":"2012-09-24T00:00:00Z","timestamp":1348444800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Italian government to Associazione Italiana Corea di Huntington-Neuromed and to IRCCS Neuromed and by Telethon","award":["GGP12218"],"award-info":[{"award-number":["GGP12218"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2012,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    <jats:bold>Background:<\/jats:bold>\n                    Huntington's disease (HD) is a devastating heredoneurodegenerative disorder associated with a wide variety of neurological and psychiatric symptoms caused by an expanded CAG repeat in the\n                    <jats:italic>HTT<\/jats:italic>\n                    gene. The expansion mutation in\n                    <jats:italic>HTT<\/jats:italic>\n                    is dominantly transmitted and codes for a protein named huntingtin (htt).\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Hypothesis:<\/jats:bold>\n                    One hypothesis, according to a multistep mechanism, is that the intergenerational transmission of the normal repeat size causes small, progressive CAG stretch elongations in the general population from one generation to another, until a critical pathological CAG repeat threshold is reached. Mutations may originate in the offspring from paternally transmitted CAG repeats, falling within an intermediate alleles (IA) range of 27 to 35 in repeat length.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Conclusions:<\/jats:bold>\n                    There has been emerging evidence that some individuals with IAs might develop an HD phenotype. This presents a challenge for genetic counseling, because these individuals are often reassured that they are \u201cdisease free.\u201d However, there are many unanswered questions related to the role of IAs in the development of the HD phenotype and in the pathogenesis of HD. \u00a9 2012 Movement Disorder Society \u00a9 2012 Movement Disorder Society\n                  <\/jats:p>","DOI":"10.1002\/mds.25172","type":"journal-article","created":{"date-parts":[[2012,9,24]],"date-time":"2012-09-24T10:29:17Z","timestamp":1348482557000},"page":"1714-1717","source":"Crossref","is-referenced-by-count":46,"title":["Huntington's disease: How intermediate are intermediate repeat 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