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They are characterized by late-onset,\nselective neuropathology, a pathogenic polyQ threshold\nand a relationship between polyQ length and disease\nprogression. Thus, molecular models of HD and\nrelated glutamine-repeat disorders must account for\nthese characteristic features. During the last three\nyears, considerable effort has been invested in the\ndevelopment of <jats:italic>in vitro<\/jats:italic> and <jats:italic>in vivo<\/jats:italic> model systems to\nstudy the mechanisms of protein aggregation in glutamine-repeat disorders and its potential effects on disease\nprogression and neurodegeneration. A selection\nof these studies is reviewed here. Furthermore, the\ncorrelation between aggregate formation and development\nof HD is discussed.<\/jats:p>","DOI":"10.1515\/bc.2000.114","type":"journal-article","created":{"date-parts":[[2004,10,11]],"date-time":"2004-10-11T16:25:41Z","timestamp":1097511941000},"page":"937-942","source":"Crossref","is-referenced-by-count":128,"title":["Protein Aggregation and Pathogenesis of Huntingtons Disease: Mechanisms and Correlations"],"prefix":"10.1515","volume":"381","author":[{"given":"Erich E.","family":"Wanker","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","reference":[{"key":"p_1","first-page":"852","volume":"13","author":"Becher M.W.","year":"1998","journal-title":"Mov. 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