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HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation
Joint Authors
Woo, So-Yeon
Kim, Ji-Yon
Hong, Young Bin
Choi, Heesun
Kim, Jisoo
Choi, Hyunjung
Mook-Jung, Inhee
Ha, Nina
Kyung, Jangbeen
Koo, Soo Kyung
Jung, Sung-Chul
Choi, Byung-Ok
Source
Issue
Vol. 2016, Issue 2016 (31 Dec. 2015), pp.1-14, 14 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-12-26
Country of Publication
Egypt
No. of Pages
14
Abstract EN
The Charcot-Marie-Tooth disease 2F (CMT2F) and distal hereditary motor neuropathy 2B (dHMN2B) are caused by autosomal dominantly inherited mutations of the heat shock 27 kDa protein 1 (HSPB1) gene and there are no specific therapies available yet.
Here, we assessed the potential therapeutic effect of HDAC6 inhibitors on peripheral neuropathy with HSPB1 mutation using in vitro model of motor neurons derived from induced pluripotent stem cells (iPSCs) of CMT2F and dHMN2B patients.
The absolute velocity of mitochondrial movements and the percentage of moving mitochondria in axons were lower both in CMT2F-motor neurons and in dHMN2B-motor neurons than those in controls, and the severity of the defective mitochondrial movement was different between the two disease models.
CMT2F-motor neurons and dHMN2B-motor neurons also showed reduced α -tubulin acetylation compared with controls.
The newly developed HDAC6 inhibitors, CHEMICAL X4 and CHEMICAL X9, increased acetylation of α -tubulin and reversed axonal movement defects of mitochondria in CMT2F-motor neurons and dHMN2B-motor neurons.
Our results suggest that the neurons derived from patient-specific iPSCs can be used in drug screening including HDAC6 inhibitors targeting peripheral neuropathy.
American Psychological Association (APA)
Kim, Ji-Yon& Woo, So-Yeon& Hong, Young Bin& Choi, Heesun& Kim, Jisoo& Choi, Hyunjung…[et al.]. 2016. HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation. Stem Cells International،Vol. 2016, no. 2016, pp.1-14.
https://search.emarefa.net/detail/BIM-1117425
Modern Language Association (MLA)
Kim, Ji-Yon…[et al.]. HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation. Stem Cells International Vol. 2016, no. 2016 (2015), pp.1-14.
https://search.emarefa.net/detail/BIM-1117425
American Medical Association (AMA)
Kim, Ji-Yon& Woo, So-Yeon& Hong, Young Bin& Choi, Heesun& Kim, Jisoo& Choi, Hyunjung…[et al.]. HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation. Stem Cells International. 2016. Vol. 2016, no. 2016, pp.1-14.
https://search.emarefa.net/detail/BIM-1117425
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1117425