Impaired ROS Scavenging System in Human Induced Pluripotent Stem Cells Generated from Patients with MERRF Syndrome

Myoclonus epilepsy associated with ragged-red fibers (MERRF) is a mitochondrial disorder characterized by myoclonus epilepsy, generalized seizures, ataxia and myopathy. MERRF syndrome is primarily due to an A to G mutation at mtDNA 8344 that disrupts the mitochondrial gene for tRNA(Lys). However, th...

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Veröffentlicht in:	Scientific reports 2016-03, Vol.6 (1), p.23661-23661, Article 23661
Hauptverfasser:	Chou, Shih-Jie, Tseng, Wei-Lien, Chen, Chien-Tsun, Lai, Yu-Fen, Chien, Chian-Shiu, Chang, Yuh-Lih, Lee, Hsin-Chen, Wei, Yau-Huei, Chiou, Shih-Hwa
Format:	Artikel
Sprache:	eng
Schlagworte:	13/100 14/28 631/532/2064/2158 631/80/304 Adolescent Antioxidants Ataxia Cardiomyocytes Cell Dedifferentiation Cell Differentiation Cells, Cultured DNA, Mitochondrial - genetics Electron microscopy Epilepsy Female Fibers Gene expression Humanities and Social Sciences Humans Induced Pluripotent Stem Cells - metabolism Induced Pluripotent Stem Cells - pathology Inhibitory postsynaptic potentials MERRF Syndrome MERRF Syndrome - metabolism MERRF Syndrome - pathology Mitochondria Mitochondria, Heart - metabolism Mitochondria, Heart - pathology Mitochondrial DNA multidisciplinary Mutation Myoclonus Myocytes, Cardiac - metabolism Myocytes, Cardiac - pathology Myopathy Neural stem cells Organelle Shape Oxygen Consumption Pluripotency Point Mutation Reactive oxygen species Reactive Oxygen Species - metabolism Science Seizures Stem cells Transmission electron microscopy tRNA Lys Ultrastructure
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