9 documents found, page 1 of 1

The stress granule protein G3BP1 alleviates spinocerebellar ataxia-associated d...

Polyglutamine diseases are a group of neurodegenerative disorders caused by an abnormal expansion of CAG repeat tracts in the codifying regions of nine, otherwise unrelated, genes. While the protein products of these genes are suggested to play diverse cellular roles, the pathogenic mutant proteins bearing an expanded polyglutamine sequence share a tendency to self-assemble, aggregate and engage in abnormal mol...

On the role of RNA binding proteins in polyglutamine diseases: from pathogenesi...

On the role of RNA binding proteins in polyglutamine diseases: from pathogenesi...

Polyglutamine (polyQ) diseases are a group of different neurodegenerative disorders characterized by an abnormal expansion of the trinucleotide cytosine-adenine-guanine (CAG) within coding regions of each disease-associated gene. The abnormal expansion translates into a protein bearing an abnormally long tract of glutamines. The expanded proteins are prone to aggregate, promote aberrant interaction with other p...

From the molecular hallmarks to motor behavior: characterization of a new trans...

Spinocerebellar ataxia type 2 (SCA2) is a rare disease with no cure, and therefore patients depend on symptomatic and supportive treatments. It is a highly debilitating disease affecting predominantly the brain with symptoms that include motor and coordination impairment. SCA2 is caused by an abnormal expansion of the CAG triplet in the coding region of the ATXN2 gene. When it has above 33 CAG repeats, it origi...

Mutant Ataxin-2 expression in aged animals aggravates neuropathological feature...

Spinocerebellar ataxia type 2 (SCA2) is a rare autosomal, dominantly inherited disease, in which the affected individuals have a disease onset around their third life decade. The molecular mechanisms underlying SCA2 are not yet completely understood, for which we hypothesize that aging plays a role in SCA2 molecular pathogenesis. In this study, we performed a striatal injection of mutant ataxin-2 mediated by le...

The stress granule protein G3BP1 alleviates spinocerebellar ataxia-associated d...

Koppenol et al. show that overexpression of G3BP1 in cell models of SCA2 and SCA3 leads to a reduction in ataxin-2 and ataxin-3 aggregation. G3BP1 lentiviral delivery reduces motor deficits and neuropathology in preclinical models, suggesting that G3BP1 may be a potential therapeutic target for polyQ disorders. Polyglutamine diseases are a group of neurodegenerative disorders caused by an abnormal expansion of ...

The cholesterol 24-hydroxylase activates autophagy and decreases mutant hunting...

Objective Compromised brain cholesterol turnover and altered regulation of brain cholesterol metabolism have been allied with some neurodegenerative diseases, including Huntington’s disease (HD). Following our previous studies in HD, in this study we aim to investigate in vitro in a neuroblastoma cellular model of HD, the effect of CYP46A1 overexpression, an essential enzyme in cholesterol metabolism, on huntin...

Formation Control Driven by Cooperative Object Tracking

In this paper we introduce a formation control loop that maximizes the performance of the cooperative perception of a tracked target by a team of mobile robots, while maintaining the team in formation, with a dynamically adjustable geometry which is a function of the quality of the target perception by the team. In the formation control loop, the controller module is a distributed non-linear model predictive co...

Falemos de casas: heterogenia e habitar - o bairro sustentável

O tema principal incide sobre a proposta de reabilitação do Bairro do Alto da Cova da Moura, que representa um dos muitos casos de bairros de génese ilegal considerados críticos, que se desenvolveram principalmente nos anos 50 e 60 do séc. XX devido às consequências do planeamento e crescimento urbano da capital e da Área Metropolitana de Lisboa. A sua proliferação estendeu-se assim na periferia da Cidade de Li...