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• W1520005101 abstract "Mitochondria are responsible for the supply of the majority of ATP in human cells via respiration and oxidative phosphorylation (OXPHOS). Mitochondria also play a central role in numerous cellular processes including energy production, intracellular Ca2+ homeostasis, biosynthesis of pyridine nucleotides and amino acids, and ┚-oxidation of fatty acids. On the contrary, mitochondria are also involved in the generation of reactive oxygen species (ROS), and initiation and execution of apoptotic cell death. Accumulating evidence show that defects in one or more of these functions may contribute to mitochondrial encephalomyopathies and other neuromuscular diseases. The term “mitochondrial encephalomyopathies” was coined by pediatric neurologists to call the attention of clinicians when the brain disease was first reported in children with mitochondrial alterations in their muscle biopsies (Shapira et al., 1977). Clinically, mitochondrial encephalomyopathies are common disorders that are a result of mutations affecting genes encoding proteins of important mitochondrial function (Leonard & Schapira, 2000a). Most mitochondrial diseases, such as mitochondrial myopathy, lactic acidosis and stroke-like episodes (MELAS), are maternally inherited and frequently manifested as encephalomyopathies (Taylor et al., 2004). Furthermore, typical clinical features of mitochondrial encephalomyopathies include weakness and retardation of the heart, skeletal muscle and brain, where mitochondria are relatively abundant. Common symptoms of mitochondrial encephalomyopathies include loss of cognitive function, sensorineural deafness, optic atrophy, fluctuating encephalopathy, ataxia, seizures, dementia, migraine, stroke-like episodes, spasticity, cardiomyopathy, proximal myopathy and exercise intolerance (Stollberger & Finsterer, 2006). Epilepsy and seizures are common features of mitochondrial dysfunction associated with mitochondrial encephalomyopathies and have been considered the most common neurological disorders. According to the electroencephalograph (EEG) and seizure procedures, epilepsy is often an important sign in the early progression of mitochondrial" @default.
• W1520005101 created "2016-06-24" @default.
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• W1520005101 date "2011-09-26" @default.
• W1520005101 modified "2023-09-26" @default.
• W1520005101 title "The Cross-Talk Between Mitochondria and the Nucleus in the Response to Oxidative Stress Associated with Mitochondrial Dysfunction in Mitochondrial Encephalomyopathies" @default.
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• W1520005101 doi "https://doi.org/10.5772/19713" @default.
• W1520005101 hasPublicationYear "2011" @default.
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