系統識別號 U0026-2801201502051000
論文名稱(中文) 微型核糖核酸miR-196a在亨丁頓氏手舞足蹈症的角色
論文名稱(英文) The role of miR-196a in the pathogenesis of Huntington's disease
校院名稱 成功大學
系所名稱(中) 基礎醫學研究所
系所名稱(英) Institute of Basic Medical Sciences
學年度 103
學期 1
出版年 104
研究生(中文) 李佳玲
研究生(英文) Chia-Ling Li
學號 s58994110
學位類別 博士
語文別 英文
論文頁數 88頁
口試委員 指導教授-楊尚訓
中文關鍵字 亨丁頓氏手舞足蹈症  微型核糖核酸-196a  基因轉殖小鼠 
英文關鍵字 Huntington's disease (HD)  miR-196a  transgenic mice 
中文摘要 亨丁頓氏手舞足蹈症(Huntington’s disease)是一種人類顯性遺傳疾病,是由於在huntingtin基因外顯子一(exon 1)有過多的CAG造成。這些突變的亨丁頓huntingtin (htt)蛋白質會形成聚集體,進而影響中樞神經系統,特別是紋狀體,也同時會造成動作失能和智能降低。至今,仍沒有藥物可以治癒亨丁頓氏手舞足蹈症。近年來,研究指出在亨丁頓氏手舞足蹈症中,轉錄失調扮演重要的因素,為了治療亨丁頓氏手舞足蹈症,已知的干擾核醣核酸(RNA interference)的技術是基因治療好方法,並且被報導可以透過後轉錄的調節進而降低突變亨丁頓。至今,尚未有文獻使用微型核糖核酸microRNA (miRNA)基因轉殖小鼠模式去探討微型核糖核酸對於亨丁頓氏手舞足蹈症的影響,此外,根據我們實驗室先前的研究發現,在亨丁頓氏手舞足蹈症中微型核糖核酸miR-196a有不正常的表現量,因此,本篇研究的研究目的為探討微型核糖核酸miR-196a參與在亨丁頓氏手舞足蹈症的角色。在細胞體外實驗結果發現,在亨丁頓氏手舞足蹈症人類胚胎腎臟或小鼠神經母細胞瘤的細胞模式中,過量表現微型核糖核酸miR-196a會降低突變亨丁頓的表現量並減少其病理性突變亨丁頓的聚集。此外,在亨丁頓氏過度表現微型核糖核酸miR-196a的基因轉殖小鼠模式中,微型核糖核酸miR-196a不僅可以減緩在紋狀體和大腦皮質病理上的突變亨丁頓聚集,例如核內、細胞核內和神經突的聚集,另一方面又可以改善亨丁頓氏手舞足蹈症晚期的動作功能。微型核糖核酸miR-196a在亨丁頓氏手舞足蹈症的有益效果可能是透過改善泛素─蛋白酶體系统(UPS)、神經膠質增生和磷酸化鈣調蛋白依賴性蛋白激酶II 。總結,我們的結果表明了微型核糖核酸miR-196a提供亨丁頓氏手舞足蹈症有益效果,透過了解微型核糖核酸miR-196a在亨丁頓氏手舞足蹈症進程中的機制,可以進而提供未來基因治療的新面向。
英文摘要 Huntington’s disease (HD) is an inherited autosomal-dominant neurodegenerative disease that is caused by expansion of CAG repeats in the exon 1 of the huntingtin gene. The aggregation of mutant huntingtin (htt) proteins affects the central nervous system, especially striatum, resulting in motor dysfunctions and cognitive declines, and at present there is no cure for HD. Recent reports have indicated that transcriptional dysregulation plays a critical role in the pathogenesis of HD. Several studies suggest that RNA interference (RNAi) is a good approach for gene therapy with regard to HD, because RNAi can decrease the expression of mutant htt at the post-transcription level. To date, no studies have used a microRNA (miRNA) transgenic mouse model to investigate the effects of miRNA on HD. Based on previous data obtained in our lab, we found that miR-196a was abnormally expressed in a HD model. Therefore, the aim of this study is to investigate the role of miR-196a in HD. Results from the in vitro study showed that overexpression of miR-196a reduced mutant htt expression and pathological aggregates in HD models of human embryonic kidney cells and mouse neuroblastoma cells. Furthermore, the results from the in vivo study showed that miR-196a not only alleviated pathological mutant htt aggregates, such as nuclear, intranuclear and neuropil aggregates in the striatum and cortex, but also improved the late-stage motor functions in HD transgenic mice overexpressing miR-196a. The beneficial effects of miR-196a in HD might be through the improvement of ubiquitin-proteasome systems (UPS), gliosis and phospho-calmodulin-dependent protein kinase II (p-CaMKII). Taken together, our results suggest that miR-196a provides beneficial effects with regard to HD. Understanding the actions of miR-196a in the progression of HD will provide further insights for the development of gene therapies for this disease.
論文目次 Abstract I
Chinese Abstract II
Acknowledgments III
Contents IV
Figure contents VII
Abbreviations IX
Chapter 1 Introduction 1
1.1 Background of Huntington’s disease (HD) 1
1.2 Gene regulation in HD 2
1.3 Biogenesis of microRNAs (miRNAs) 3
1.3.1 Generation of primary miRNAs (pri-miRNAs) 4
1.3.2 Generation of precursor miRNAs (pre-miRNAs) 4
1.3.3 The translocation of precursor miRNAs (pre-miRNAs) 4
1.3.4 Generation of mature miRNAs: cytoplasmic processing of pre-miRNAs 5
1.3.5 Generation of mature miRNAs: formation of miRNA- RISC complex (miRISC) 5
1.4 Actions of miRNA-mediated gene silencing 6
1.5 Cellular functions of miRNAs 7
1.6 MiRNAs in the brain 7
1.7 MiRNAs and HD 9
1.8 The significance of this study 11
Chapter 2 Objective and specific aims 12
Chapter 3 Materials and Methods 13
3.1 DNA construction 13
3.2 DNA transformation 13
3.3 Plasmid DNA extraction 14
3.4 Lentiviruses preparation 14
3.5 Cell culture, transfection and in vitro analyses 14
3.6 Subculture of the cells 15
3.7 Freezing of the cells 16
3.8 Thawing out of the cells 16
3.9 Generation of transgenic mice 17
3.10 Genotyping for transgenic mice 17
3.11 RNA preparation and real-time polymerase chain reaction (real-time PCR) for gene expression 18
3.12 Western blotting analysis 19
3.13 Immunohistochemical analysis 22
3.14 Behavioral tests 23
3.15 Statistical analysis 24
Chapter 4 Results 25
4.1 miR-196a suppressed the mutant htt expression in vitro 25
4.2 High expression of mature miR-196a after FUmiR196a transfection in vitro 26
4.3 High expression of mature miR-196a in miR-196a transgenic mice 26
4.4 Improvements at the molecular level in D-Tg transgenic mice 27
4.5 Improvements at the pathological level in D-Tg transgenic mice 28
4.6 Improvements in the motor function in D-Tg transgenic mice 28
4.7 Improvements in the ubiquitin-proteasome system (UPS), gliosis and calmodulin-dependent protein kinase II (CaMKII) phosphorylation in D-Tg transgenic mice 29
Chapter 5 Discussion 31
Chapter 6 Conclusions 38
Chapter 7 References 39
Chapter 8 Figures 65
Publications 88
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