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Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3

Laço, Mário N.; Cortes, Luísa; Travis, Sue M.; Paulson, Henry L.; Rego, A. Cristina

Alterations in the ubiquitin-proteasome system (UPS) have been reported in several neurodegenerative disorders characterized by protein misfolding and aggregation, including the polylgutamine diseases. Machado-Joseph disease (MJD) or Spinocerebellar Ataxia type 3 is caused by a polyglutamine-encoding CAG expansion in the ATXN3 gene, which encodes a 42 kDa deubiquitinating enzyme (DUB), ataxin-3. We investigated...

Date: 2012   |   Origin: Estudo Geral - Universidade de Coimbra
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