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Inventi Impact: Genetics

Articles

  • Inventi:hgt/22368/17
    HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived\nfrom the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation
    01-Jan-1970 Research 2017 : April - June
    Ji-Yon Kim, So-Youn Woo, Young Bin Hong, Heesun Choi, Jisoo Kim, Hyunjung Choi, Inhee Mook-Jung, Nina Ha, Jangbeen Kyung, Soo Kyung Koo, Sung-Chul Jung, Byung-Ok Choi

    The Charcot-Marie-Tooth disease 2F (CMT2F) and distal hereditary motor neuropathy 2B (dHMN2B) are caused by autosomal\ndominantly inherited mutations of the heat shock 27 kDa protein 1 (HSPB1) gene and there are no specific therapies available yet.\nHere, we assessed the potential therapeutic effect of HDAC6 inhibitors on peripheral neuropathy with HSPB1 mutation using in\nvitro model of motor neurons derived from induced pluripotent stem cells (iPSCs) of CMT2F and dHMN2B patients. The absolute\nvelocity of mitochondrial movements and the percentage of moving mitochondria in axons were lower both in CMT2F-motor\nneurons and in dHMN2B-motor neurons than those in controls, and the severity of the defective mitochondrial movement was\ndifferent between the two disease models. CMT2F-motor neurons and dHMN2B-motor neurons also showed reduced

    How to Cite this Article
    CC Compliant Citation: Ji-Yon Kim, So-Youn Woo, Young Bin Hong,\net al., â??HDAC6 Inhibitors Rescued the Defective Axonal\nMitochondrial Movement in Motor Neurons Derived from the\nInduced Pluripotent Stem Cells of Peripheral Neuropathy Patients\nwith HSPB1 Mutation,â? Stem Cells International, vol. 2016, Article\nID 9475981, 14 pages, 2016. doi:10.1155/2016/9475981, http://\ncreativecommons.org/licenses/by/4.0/.
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