6 documents found, page 1 of 1
MPV17 Mutations Are Associated With a Quiescent Energetic Metabolic Profile
Jacinto, S; Guerreiro, P; Machado de Oliveira, R; Cunha-Oliveira, T; Santos, MJ; Grazina, M; Rego, AC; Outeiro, TFMutations in the MPV17 gene are associated with hepatocerebral form of mitochondrial depletion syndrome. The mechanisms through which MPV17 mutations cause respiratory chain dysfunction and mtDNA depletion is still unclear. The MPV17 gene encodes an inner membrane mitochondrial protein that was recently described to function as a non-selective channel. Although its exact function is unknown, it is thought to be...
MPV17 Mutations Are Associated With a Quiescent Energetic Metabolic Profile
Jacinto, S; Guerreiro, P; de Oliveira, RM; Cunha-Oliveira, T; Santos, MJ; Grazina, M; Rego, AC; Outeiro, TFMutations in the MPV17 gene are associated with hepatocerebral form of mitochondrial depletion syndrome. The mechanisms through which MPV17 mutations cause respiratory chain dysfunction and mtDNA depletion is still unclear. The MPV17 gene encodes an inner membrane mitochondrial protein that was recently described to function as a non-selective channel. Although its exact function is unknown, it is thought to be...
The NAD+-dependent deacetylase SIRT2 attenuates oxidative stress and mitochondr...
Lemos, V; de Oliveira, RM; Naia, L; Szegö, É; Ramos, E; Pinho, S; Magro, F; Cavadas, C; Rego, AC; Outeiro, TF; Gomes, PInsulin resistance is a major predictor of the development of metabolic disorders. Sirtuins (SIRTs) have emerged as potential targets that can be manipulated to counteract age-related diseases, including type 2 diabetes. SIRT2 has been recently shown to exert important metabolic effects, but whether SIRT2 regulates insulin sensitivity in hepatocytes is currently unknown. The aim of this study is to investigate ...
Bioenergetic dysfunction in Huntington's disease human cybrids
Ferreira, IL; Cunha-Oliveira, T; Nascimento, MV; Ribeiro, M; Proença, MT; Januário, C; Oliveira, CR; Rego, ACIn this work we studied the mitochondrial-associated metabolic pathways in Huntington's disease (HD) versus control (CTR) cybrids, a cell model in which the contribution of mitochondrial defects from patients is isolated. HD cybrids exhibited an interesting increase in ATP levels, when compared to CTR cybrids. Concomitantly, we observed increased glycolytic rate in HD cybrids, as revealed by increased lactate/p...
Mitochondrial-dependent apoptosis in Huntington's disease human cybrids
Ferreira, IL; Nascimento, MV; Ribeiro, MH; Almeida, S; Cardoso, SM; Grazina, M; Pratas, J; Santos, MJ; Januário, C; Oliveira, CR; Rego, ACWe investigated the involvement of mitochondrial-dependent apoptosis in Huntington's disease (HD) vs. control (CTR) cybrids, obtained from the fusion of human platelets with mitochondrial DNA-depleted NT2 cells, and further exposed to 3-nitropropionic acid (3-NP) or staurosporine (STS). Untreated HD cybrids did not exhibit significant modifications in the activity of mitochondrial respiratory chain complexes I-...
O Carvedilol Protege Mitocôndrias Cardíacas Isquémicas de Lesões Induzidas Por ...
Carreira, R; Duarte, AI; Monteiro, P; Santos, MS; Rego, AC; Oliveira, CR; Gonçalves, L; Providência, LAIschemia negatively affects mitochondrial function by inducing the mitochondrial permeability transition (MPT). The MPT is triggered by oxidative stress, which occurs in mitochondria during ischemia as a result of diminished antioxidant defenses and increased reactive oxygen species production. It causes mitochondrial dysfunction and can ultimately lead to cell death. Therefore, drugs able to minimize mitochond...