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Absence of ataxin-3 leads to enhanced stress response in C. elegans
Rodrigues, Ana João; Carvalho, Andreia Neves; Castro, Andreia Cristiana Teixeira de; Rokka, Anne; Corthals, Gary; Logarinho, Elsa; Maciel, P.Ataxin-3, the protein involved in Machado-Joseph disease, is able to bind ubiquitylated substrates and act as a deubiquitylating enzyme in vitro, and it has been involved in the modulation of protein degradation by the ubiquitinproteasome pathway. C. elegans and mouse ataxin-3 knockout models are viable and without any obvious phenotype in a basal condition however their phenotype in stress situations has never...
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...
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