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Ataxin-3 plays a role in mouse myogenic differentiation through regulation of i...
Costa, Maria do Carmo; Bajanca, Fernanda; Rodrigues, Ana Jo??o; Tom??, Ricardo J.; Corthals, Gary; Ribeiro, Sandra Macedo; Paulson, Henry L.BACKGROUND: During myogenesis several transcription factors and regulators of protein synthesis and assembly are rapidly degraded by the ubiquitin-proteasome system (UPS). Given the potential role of the deubiquitinating enzyme (DUB) ataxin-3 in the UPS, and the high expression of the murine ataxin-3 homolog in muscle during embryogenesis, we sought to define its role in muscle differentiation. METHODOLOGY/PRIN...
Nucleocytoplasmic shuttling activity of ataxin-3
Maciel, P.; Ribeiro, Sandra Macedo; Cortes, Lu??sa; Carvalho, Ana Lu??saSpinocerebellar ataxia type-3, also known as Machado-Joseph Disease (MJD), is one of many inherited neurodegenerative disorders caused by polyglutamine-encoding CAG repeat expansions in otherwise unrelated genes. Disease protein misfolding and aggregation, often within the nucleus of affected neurons, characterize polyglutamine disorders. Several evidences have implicated the nucleus as the primary site of path...
Towards a structural understanding of the fibrillization pathway in Machado-Jos...
Gales, Luís; Cortes, Luísa; Almeida, Carla; Melo, Carlos V.; Costa, Maria do Carmo; Maciel, P.; Clarke, David T.; Damas, Ana MargaridaMachado-Joseph’s disease is caused by a CAG trinucleotide repeat expansion that is translated into an abnormally long polyglutamine tract in the protein ataxin-3. Except for the polyglutamine region, proteins associated with polyglutamine diseases are unrelated, and for all of these diseases aggregates containing these proteins are the major components of the nuclear proteinaceous deposits found in the brain. A...
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