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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...


The human spindle assembly checkpoint protein Bub3 is required for the establis...

Logarinho, Elsa; Resende, Tatiana; Torres, C.; Bousbaa, Hassan

The spindle assembly checkpoint monitors the status of kinetochore–microtubule (K-MT) attachments and delays anaphase onset until full metaphase alignment is achieved. Recently, the role of spindle assembly checkpoint proteins was expanded with the discovery that BubR1 and Bub1 are implicated in the regulation of K-MT attachments. One unsolved question is whether Bub3, known to form cell cycle constitutive comp...


NEDD8: A new ataxin-3 interactor

Ferro, Anabela; Carvalho, Ana Luísa; Teixeira-Castro, Andreia; Almeida, Carla; Tomé, Ricardo J.; Cortes, Luísa; Rodrigues, Ana-João; Logarinho, Elsa

Machado-Joseph disease (MJD/SCA3) is an autosomal dominant neurodegenerative disease caused by the expansion of a CAG tract in the coding portion of the ATXN3 gene. The presence of ubiquitin-positive aggregates of the defective protein in affected neurons is characteristic of this and most of the polyglutamine disorders. Recently, the accumulation of the neural precursor cell expressed developmentally downregul...


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