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MPV17 mutations are associated with a quiescent energetic metabolic profile

Jacinto, Sandra; Guerreiro, Patrícia; de Oliveira, Rita Machado; Cunha-Oliveira, Teresa; Santos, Maria João; Grazina, Manuela; Rego, Ana Cristina

© 2021 Jacinto, Guerreiro, de Oliveira, Cunha-Oliveira, Santos, Grazina, Rego and Outeiro. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordan...


Neuroprotection of retinal ganglion cells by the sigma-1 receptor agonist prido...

Geva, Michal; Gershoni-Emek, Noga; Naia, Luana; Ly, Philip; Mota, Sandra; Rego, Ana Cristina; Hayden, Michael R.; Levin, Leonard A.

Optic neuropathies such as glaucoma are characterized by retinal ganglion cell (RGC) degeneration and death. The sigma-1 receptor (S1R) is an attractive target for treating optic neuropathies as it is highly expressed in RGCs, and its absence causes retinal degeneration. Activation of the S1R exerts neuroprotective effects in models of retinal degeneration. Pridopidine is a highly selective and potent S1R agoni...


Mitochondrial and redox-based therapeutic strategies in Huntington's disease

Fão, Lígia; Rego, Ana Cristina

Significance: The molecular processes that determine Huntington's disease (HD) pathogenesis are not yet fully understood, and until now no effective neuroprotective therapeutic strategies have been developed. Mitochondria are one of most important organelles required for neuronal homeostasis, by providing metabolic pathways relevant for energy production, regulating calcium homeostasis, or controlling free radi...


Calcium Signaling in Aging and Neurodegenerative Diseases 2019

Cortes, Luísa; Malva, João; Rego, Ana Cristina; Pereira, Cláudia F.

The European Calcium Society (ECS) workshop, which is held every 2 years, is a dedicated meeting of scientists interested in the elucidation of the action of calcium binding, calcium signaling and the study of proteins and organelles, such as mitochondria and endoplasmic reticulum, thereby involved, either in health and disease conditions. The 8th edition of the ECS workshop was organized by a group of research...


Microbial BMAA elicits mitochondrial dysfunction, innate immunity activation, a...

Silva, Diana F.; Candeias, Emanuel; Esteves, Ana Raquel Fernandes; Magalhães, João Duarte; Ferreira, I. Luísa; Nunes-Costa, Daniela; Rego, Ana Cristina

Background: After decades of research recognizing it as a complex multifactorial disorder, sporadic Alzheimer’s disease (sAD) still has no known etiology. Adding to the myriad of different pathways involved, bacterial neurotoxins are assuming greater importance in the etiology and/or progression of sAD. β-N-Methylamino-L-alanine (BMAA), a neurotoxin produced by some microorganisms namely cyanobacteria, was prev...


Shaping the Nrf2-ARE-related pathways in Alzheimer's and Parkinson's diseases

Fão, Lígia; Mota, Sandra; Rego, Ana Cristina

A failure in redox homeostasis is a common hallmark of Alzheimer's Disease (AD) and Parkinson's Disease (PD), two age-dependent neurodegenerative disorders (NDD), causing increased oxidative stress, oxidized/damaged biomolecules, altered neuronal function and consequent cell death. Activation of nuclear factor erythroid 2-related factor 2 (Nrf2), a redox-regulated transcription factor, results in upregulation o...


Chronic stress triggers divergent dendritic alterations in immature neurons of ...

Dioli, Chrysoula; Patrício, Patrícia; Sousa, Nuno; Kokras, Nikolaos; Dalla, Christina; Guerreiro, Sara; Santos-Silva, Miguel A.; Rego, Ana Cristina

Chronic stress, a suggested precipitant of brain pathologies, such as depression and Alzheimer's disease, is known to impact on brain plasticity by causing neuronal remodeling as well as neurogenesis suppression in the adult hippocampus. Although many studies show that stressful conditions reduce the number of newborn neurons in the adult dentate gyrus (DG), little is known about whether and how stress impacts ...


Chronic stress triggers divergent dendritic alterations in immature neurons of ...

Dioli, Chrysoula; Patrício, Patrícia; Sousa, Nuno; Kokras, Nikolaos; Dalla, Christina; Guerreiro, Sara; Santos-Silva, Miguel A.; Rego, Ana Cristina

Chronic stress, a suggested precipitant of brain pathologies, such as depression and Alzheimer's disease, is known to impact on brain plasticity by causing neuronal remodeling as well as neurogenesis suppression in the adult hippocampus. Although many studies show that stressful conditions reduce the number of newborn neurons in the adult dentate gyrus (DG), little is known about whether and how stress impacts ...


Coriolus versicolor biomass increases dendritic arborization of newly-generated...

Ferreiro, Elisabete; Pita, Inês R.; Mota, Sandra; Valero, Jorge; Ferreira, Nuno R.; Fernandes, Tito; Calabrese, Vittorio; Fontes-Ribeiro, Carlos A.

Brain cognitive reserve refers to the ability of the brain to manage different challenges that arise throughout life, making it resilient to neuropathology. Hippocampal adult neurogenesis has been considered to be a relevant contributor for brain cognitive reserve and brain plasticity. Coriolus versicolor (CV), a common healthful mushroom, has been receiving increasing attention by its antitumoral, anti-inflamm...


Dominant-Negative Effects of Adult-Onset Huntingtin Mutations Alter the Divisio...

Lopes, Carla; Aubert, Sophie; Bourgois-Rocha, Fany; Barnat, Monia; Rego, Ana Cristina; Déglon, Nicole; Perrier, Anselme L.; Humbert, Sandrine

Mutations of the huntingtin protein (HTT) gene underlie both adult-onset and juvenile forms of Huntington's disease (HD). HTT modulates mitotic spindle orientation and cell fate in mouse cortical progenitors from the ventricular zone. Using human embryonic stem cells (hESC) characterized as carrying mutations associated with adult-onset disease during pre-implantation genetic diagnosis, we investigated the infl...


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