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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Vydáno v:Sci Rep
Hlavní autoři: 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
Médium: Artigo
Jazyk:Inglês
Vydáno: Nature Publishing Group 2016
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Article
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC4812254/
https://ncbi.nlm.nih.gov/pubmed/27025901
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep23661
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