... access for this article was enabled by the participation of Newcastle University in an all-inclusive Read & Publish agreement with Portland Press and the Biochemical Society under a transformative agreement with JISC. DNA damage DNA replication and recombination mitochondrial dysfunction...

...Valeria Di Leo; Tiago M. Bernardino Gomes; Amy E. Vincent Mitochondrial dysfunction in skeletal muscle fibres occurs with both healthy aging and a range of neuromuscular diseases. The impact of mitochondrial dysfunction in skeletal muscle and the way muscle fibres adapt to this dysfunction...

... The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society 2021 calcitriol calcium homoeostasis frataxin mitochondrial dysfunction Friedreich ataxia (FA) is caused by decreased expression of the mitochondrial protein frataxin due to large expansions of GAA...

.... This review aims to act as an interface between the clinical and basic research underpinning our knowledge of OXPHOS complex and ribosome assembly, and the dysfunction of this process in mitochondrial disease. mitochondria mitochondrial dysfunction mitochondrial respiration mutation oxidative...

... equally to this work. 11 5 2020 20 7 2020 3 8 2020 © 2020 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society 2020 mitochondrial dysfunction oxidative stress protein import protein misfolding translation As a eukaryotic cell...

... be prevented by cyclosporine A (a blocker of mitochondrial permeability transition pore). These results indicate that iron cytotoxicity is mediated by α-synuclein acting on mitochondria. Likewise siRNA mediated knock-down of Parkin causes an accumulation of α-synuclein accompanied by mitochondrial dysfunction...

... and cytotoxicity revealed differences between the assessed α-syn species. Only oligomers induced mitochondrial dysfunction in astrocytes and significantly increased extracellular hydrogen peroxide production by these cells. Besides, TNF-α and IL-1β (interleukin 1β) expression presented different kinetics...

...Juvid Aryaman; Iain G. Johnston; Nick S. Jones Mitochondrial dysfunction is involved in a wide array of devastating diseases, but the heterogeneity and complexity of the symptoms of these diseases challenges theoretical understanding of their causation. With the explosion of omics data, we have...

...). Given that mitochondrial dysfunction has been identified in fibroblasts, PBMC and brain samples from carriers as well as in animal models of the premutation and that mitochondria are at the center of intermediary metabolism, the aim of the present study was to provide a complete view of the metabolic...

... ]. inborn error of metabolism mitochondrial dysfunction mouse genetics mtDNA Succinate-CoA ligase (SUCL), also known as succinyl coenzyme A synthetase, or succinate thiokinase is a heterodimer enzyme composed of an invariant α-subunit encoded by SUCLG1 and a substrate-specific β-subunit encoded...

... of mitochondrial dysfunction in fatty-acid-induced β-cell failure. We have used state-of-the-art extracellular flux technology to functionally probe mitochondrial energy metabolism in intact INS-1E insulinoma cells in real-time. We show that 24-h palmitate exposure at high glucose attenuates the glucose...

... engagement, the resulting apoptotic mechanisms and the protective pharmacological effect of methazolamide. cerebral amyloid angiopathy familial Alzheimer’s disease methazolamide mitochondrial dysfunction post-translational modification Biochemical analyses after sequential tissue extraction...

... and in vivo level. © The Authors Journal compilation © 2010 Biochemical Society 2010 Alzheimer's disease amyloid-binding alcohol dehydrogenase (ABAD) cyclophilin D (CypD) intracellular amyloid-β peptide (Aβ) mitochondrial dysfunction mitochondrial permeability transition pore AD...

...Marina A. Schwab; Sven W. Sauer; Jürgen G. Okun; Leo G. J. Nijtmans; Richard J. T. Rodenburg; Lambert P. van den Heuvel; Stefan Dröse; Ulrich Brandt; Georg F. Hoffmann; Henk Ter Laak; Stefan Kölker; Jan A. M. Smeitink Mitochondrial dysfunction during acute metabolic crises is considered...