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Development and characterization of PHB-HV based 3D scaffolds for a tissue engi...
Samy, S. M.; Silva, Nuno; Pinto, Luísa; Castro, Andreia Cristiana Teixeira de; Almeida, Hugo Leite; Almeida, Armando; Sousa, Nuno; Salgado, António J.Spinal cord injury (SCI) leads to devastating neurological deficits. Several tissue engineering (TE)- based approaches have been investigated for repairing this condition. Poly (3-hydroxybutyrateco- 3-hydroxyvalerate) (PHB-HV) is found to be particularly attractive for TE applications due to its properties, such as biodegradability, biocompatibility, thermoplasticity and piezoelectricity. Hence, this report add...
Identification of modulators of ataxin-3 proteotoxicity in animal models of Mac...
Castro, Andreia Cristiana Teixeira deTese doutoramento em Ciências da Saúde (especialidade de Ciências da Saúde) ; At least nine human neurodegenerative diseases are caused by the expansion of CAG repeats within otherwise unrelated genes. In these diseases, including Machado-Joseph disease (MJD), polyglutamine (polyQ) expansions cause the appearance of misfolded protein species, that ultimately lead to the formation of aggregates and neuronal lo...
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...
Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the ...
Castro, Andreia Cristiana Teixeira de; Ailion, Michael; Jalles, Ana; Brignull, Heather; Vilaça, João L.; Dias, N. S.; Rodrigues, Pedro L.The risk of developing neurodegenerative diseases increases with age. Although many of the molecular pathways regulating proteotoxic stress and longevity are well characterized, their contribution to disease susceptibility remains unclear. In this study, we describe a new Caenorhabditis elegans model of Machado–Joseph disease pathogenesis. Pan-neuronal expression of mutant ATXN3 leads to a polyQ-length dependen...
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