Notably, several mitochondrial defects have been identified in central nervous system disorders. Membrane leakage and electrolyte imbalances, pro-apoptotic pathway activation, and mitophagy are among the mechanisms implicated in the pathogenesis of neurodegenerative diseases, such as Alzheimer's, Parkinson's...
Mitochondrial disorders in the nervous system. Mitochondrial diseases (encephalomyopathies) have traditionally been ascribed to defects of the respiratory chain, which has helped researchers explain the... S Dimauro,EA Schon - 《Annual Review of Neuroscience》 被引量: 757发表: 2008年 Monogenic mito...
Neurodegenerative diseases are a large group of disabling disorders of the nervous system, characterized by the relative selective death of neuronal subtypes. In most cases, there is overwhelming evidence of impaired mitochondrial function as a causative factor in these diseases. More recently, evidence...
Defects of mitochondrial DNA (mtDNA) maintenance and mitochondrial translation (mtDNA gene expression defects) are the most common causes of mitochondrial disease, which was revealed by next-generation genetic tools. These disorders can be caused by mutations in nuclear or mtDNA genes. ...
Burte F, Carelli V, Chinnery PF, Yu-Wai-Man P (2015) Disturbed mitochondrial dynamics and neurodegenerative disorders. Nat Rev Neurol 11(1):11–24 Article CAS PubMed Google Scholar Archer SL (2013) Mitochondrial dynamics–mitochondrial fission and fusion in human diseases. N Engl J Med 369...
Mitochondria are essential organelles involved in cellular energy production. Changes in mitochondrial function can lead to dysfunction and cell death in aging and age-related disorders. Recent research suggests that mitochondrial dysfunction is closely
Mitochondrial Disorders (MD) include a heterogeneous group of inherited disorders due to molecular defects mainly affecting the mitochondrial oxidative phosphorylation system. Because the respiratory chain is under control of two different genomes (nuclear DNA-nDNA and mitochondrial DNA-mtDNA), mitochondrial...
Virtually all cells in humans depend on mitochondrial oxidative phosphorylation to generate energy, accounting for the remarkable diversity of clinical disorders associated with mitochondrial DNA mutations. However, certain tissues are particularly susceptible to mitochondrial dysfunction, resulting in recognizable...
Myocardial metabolic disorders are largely affected by the TCA cycle, OXPHOS and FAO. During pathological heart remodeling, cardiac metabolism is reprogrammed to increase reliance on glucose and significantly increase glycolysis, whereas OXPHOS and FAO are downregulated.28Increased glycolysis is associated wi...
Mobile mitochondria can become stationary or pause in regions that have a high metabolic demand and can move again rapidly in response to physiological changes. Defects in mitochondrial transport are implicated in the pathogenesis of several major neurological disorders. Research into the mechanisms that...