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
المؤلفون المشاركون
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
المصدر
العدد
المجلد 2016، العدد 2016 (31 ديسمبر/كانون الأول 2015)، ص ص. 1-14، 14ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2016-12-26
دولة النشر
مصر
عدد الصفحات
14
الملخص 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.
نمط استشهاد جمعية علماء النفس الأمريكية (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
نمط استشهاد الجمعية الأمريكية للغات الحديثة (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
نمط استشهاد الجمعية الطبية الأمريكية (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
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1117425
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
تقوم هذه الخدمة بالتحقق من التشابه أو الانتحال في الأبحاث والمقالات العلمية والأطروحات الجامعية والكتب والأبحاث باللغة العربية، وتحديد درجة التشابه أو أصالة الأعمال البحثية وحماية ملكيتها الفكرية. تعرف اكثر