{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T18:24:10Z","timestamp":1791224650594,"version":"4.1.0"},"reference-count":48,"publisher":"Rockefeller University Press","issue":"1","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2006,7,3]]},"abstract":"<jats:p>The pathogenesis of spinocerebellar ataxia type 7 and other neurodegenerative polyglutamine (polyQ) disorders correlates with the aberrant accumulation of toxic polyQ-expanded proteins in the nucleus. Promyelocytic leukemia protein (PML) nuclear bodies are often present in polyQ aggregates, but their relation to pathogenesis is unclear. We show that expression of PML isoform IV leads to the formation of distinct nuclear bodies enriched in components of the ubiquitin-proteasome system. These bodies recruit soluble mutant ataxin-7 and promote its degradation by proteasome-dependent proteolysis, thus preventing the aggregate formation. Inversely, disruption of the endogenous nuclear bodies with cadmium increases the nuclear accumulation and aggregation of mutant ataxin-7, demonstrating their role in ataxin-7 turnover. Interestingly, \u03b2-interferon treatment, which induces the expression of endogenous PML IV, prevents the accumulation of transiently expressed mutant ataxin-7 without affecting the level of the endogenous wild-type protein. Therefore, clastosomes represent a potential therapeutic target for preventing polyQ disorders.<\/jats:p>","DOI":"10.1083\/jcb.200511045","type":"journal-article","created":{"date-parts":[[2006,7,5]],"date-time":"2006-07-05T20:54:25Z","timestamp":1152132865000},"page":"65-76","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":86,"title":["PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins"],"prefix":"10.1083","volume":"174","author":[{"given":"Alexandre","family":"Janer","sequence":"first","affiliation":[{"name":"1Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale U679, Neurologie et The\u0301rapeutique Expe\u0301rimentale, 75651 Paris Cedex 13, France"},{"name":"2Ho\u0302pital de la Pitie\u0301-Salpe\u0302trie\u0300re, 75651 Paris Cedex 13, France"},{"name":"3Faculte\u0301 de Me\u0301decine, Universite\u0301 Pierre et Marie Curie, 75651 Paris Cedex 13, 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