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研究生:張雅筑
研究生(外文):Chang, Ya Chu
論文名稱:VCP於 IBMPFD致病機轉中扮演重要角色並協助已分化細胞維持其細胞核結構與功能之完整性
論文名稱(外文):VCP contributes to IBMPFD pathogenesis and the maintenance of post-mitotic nuclear structure and function
指導教授:桑自剛
指導教授(外文):Sang, Tzu Kang
學位類別:博士
校院名稱:國立清華大學
系所名稱:生物科技研究所
學門:生命科學學門
學類:生物科技學類
論文種類:學術論文
論文出版年:2016
畢業學年度:104
語文別:英文
論文頁數:159
中文關鍵詞:IBMPFD疾病VCP蛋白神經退化細胞核
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Valosin-containing protein(簡稱VCP)為一種AAA (ATPase associated with diverse cellular activities) ATPase,透過水解ATP所產生之能量參與細胞中多種生理活動,包含泛素標定蛋白的降解(ubiquitin-dependent protein degradation)、胞器的形成、DNA損傷反應(DNA damage response),以及細胞週期的調控。透過家族遺傳性inclusion body myopathy with Paget disease of bone and frontotemporal dementia (簡稱IBMPFD)的研究發現,VCP突變與此疾病的產生有關;然而突變VCP是經由何種途徑致病卻仍不清楚。我們將果蠅的VCP同源蛋白,TER94,做了相對應於人類VCP蛋白上與IBMPFD疾病有關的相同突變,並表現於果蠅的特定組織中,藉此建立果蠅的IBMPFD疾病研究模式。我們證實了此一模式能夠重現IBMPFD疾病中多重生理系統失調的現象,此外,我們的研究顯示IBMPFD致病突變的本質為dominant-active,意即突變導致TER94蛋白其ATPase活性過度活躍而造成細胞內ATP含量失衡,進一步擾亂細胞中其他依賴ATP來進行的生理活動並致使對能量具有高度需求的組織產生漸行性退化。除了揭露IBMPFD的致病機轉,我們在研究中也發現TER94尚未被提及的潛在功能:協助維持已分化細胞其細胞核之結構與功能。根據先前的研究,已知VCP參與細胞分裂後核膜重組的過程;然而我們觀察到,在已分化完全、不再進行細胞分裂的細胞中表現dominant-negative mutant TER94會造成核膜異常的增大。透過分析細胞核的形態變化與TER94在細胞中分布位置之間的關聯性,我們認為TER94在細胞核內的功能對於維持正常的細胞核形態是不可或缺的。此外,我們觀察到在TER94功能受損時,細胞核內的泛素標定蛋白會大量堆積並與TER94結合在一起,這一現象指出細胞核的異常擴增可能導因於dominant-negative TER94使細胞核內缺乏能正常作用的TER94 complex,進而使核內蛋白質的降解受到阻礙。有趣的是,我們發現dominant-negative TER94不但能影響細胞核的形態,同時會改變細胞核內染色質(chromatin)的分布與組蛋白(histone)的修飾,意味著TER94也許能經由對細胞核結構的調控來影響細胞核的功能,特別是基因的表現。
Valosin-containing protein (VCP), an AAA (ATPase associated with diverse cellular activities) ATPase, has been reported to participate in various biological events, including ubiquitin-dependent protein degradation, organelle biogenesis, DNA damage response, and cell cycle regulation. Studies of familial inclusion body myopathy with Paget disease of bone and frontotemporal dementia (IBMPFD) have linked this disease to VCP mutations. We established a Drosophila IBMPFD model by tissue-specific expression of TER94 (Drosophila VCP homolog) mutants that correspond to VCP disease alleles, and by which to demonstrate that our model could recapitulate the multi-system disorders characteristic of IBMPFD. Furthermore, we provided evidence showing that IBMPFD-causing mutants are dominant-active alleles and may cause an imbalance of cellular ATP, which in turn perturb ATP-dependent processes within cells and progressively lead to the degeneration of tissues that demand higher energy expenditure. Apart from revealing the underlying mechanism of IBMPFD pathogenesis, we identified a potential role of TER94 in maintaining post-mitotic nuclear structure. It is already known that VCP is involved in nuclear envelope reformation after mitosis; however, we observed the size of the nuclei remarkably increased in non-dividing cells expressing TER94 dominant-negative mutants. Relationship between the nuclear morphology and cellular localization of TER94 illustrated that the activity of TER94 hexamer in the nucleus is required for maintaining a normal nuclear size. In addition, we found that ubiquitinated proteins accumulated and were associated with TER94 in the nucleus as the function of TER94 being disrupted, suggesting that TER94 might have a role in nuclear protein quality control and the nuclear enlargement could be attributed to aberrant nuclear protein degradation. Interestingly, the nuclear expansion induced by dominant-negative mutant TER94 were accompanied with altered chromatin distribution and histone modification, implicating that the gene expression profile could be affected due to the change in nuclear morphology. This also indicates that the maintenance of nuclear structure is important for the nucleus to function properly.
CHAPTER 1 – Introduction
1.1 The identification of VCP 1
1.2 Molecular properties of VCP 4
1.2.1 The N domain 5
1.2.2 The AAA domains 6
1.2.3 ATP hydrolysis and the conformational changes in VCP 7
1.3 Biological functions of VCP 8
1.4 The implication of VCP in diseases 12
CHAPTER 2 – The Establishment of Drosophila IBMPFD Model
2.1 Introduction 16
2.2 Results 18
2.2.1 Expression of TER94 IBMPFD mutants results in muscle disorganization 18
2.2.2 Expressing TER94 IBMPFD mutants in Drosophila brain causes structural abnormality and learning deficit 20
2.2.3 Expressing TER94 IBMPFD mutants in Drosophila eye induces photoreceptor degeneration 22
2.3 Discussion 25
CHAPTER 3 – Investigation of the Pathogenic Mechanisms Underlying IBMPFD
3.1 Introduction 27
3.2 Results 28
3.2.1 Formation of TER94-containing aggregates is not directly linked to photoreceptor degeneration 28
3.2.2 TER94 IBMPFD mutant-induced neurodegeneration is not associated with UPS impairment 29
3.2.3 TER94 IBMPFD mutant-induced neurodegeneration is not due to ERAD dysfunction 30
3.2.4 Formation of hexamer is necessary for TER94 IBMPFD mutants-induced neurodegeneration 33
3.2.5 TER94 IBMPFD mutations are dominant active alleles 35
3.2.6 TER94 IBMPFD mutants exhibit elevated ATPase activity and cause a reduction in cellular ATP 36
3.2.7 Energy expenditure is sssociated with IBMPFD pathogenesis in fly model 37
3.2.8 Downregulation of Drosophila plip suppresses IBMPFD mutants-induced neurodegeneration 39
3.3 Discussion 41
CHAPTER 4 – VCP Contributes to the Maintenance of Post-Mitotic Nuclear Structure and Function
4.1 Introduction 46
4.1.1 Post-mitotic nuclear structure might need to be constantly maintained 46
4.1.2 Nuclear aberration and diseases 47
4.2 Results 49
4.2.1 The abolishment of TER94 function leads to nuclear expansion 49
4.2.2 Down-regulation of TER94 does not induce nuclear structure aberration 53
4.2.3 The overall cell volume does not increase in proportion to the nuclear enlargement 53
4.2.4 Misregulation of lipid synthesis might not be the main cause of TER94K2A-induced nuclear expansion 55
4.2.5 Down-regulation of Lamin or Ntf2 could moderately suppress the nuclear expansion caused by TER94K2A 57
4.2.6 The nucleocytoplasmic trafficking seemed not to be affected by TER94K2A 59
4.2.7 The disruption of TER94-dependent nuclear protein degradation might be the underlying cause of nuclear expansion 61
4.2.8 The nuclear expansion has an influence on chromatin distribution and gene expression 65
4.3 Discussion 69
CHAPTER 5 – Conclusion Remarks 75
CHAPTER 6 – Materials and Methods 77
6.1 Drosophila genetics and molecular biology 77
6.2 Antibody production and immunohistochemistry 78
6.3 Behavior assays 79
6.4 Immunoblotting and immunoprecipitation 80
6.5 RT-PCR 81
6.6 Quantification of rhabdomeres in adult retina 81
6.7 Dietary restriction 82
6.8 Heat shock conditions 82
6.9 ATP level measurement 82
6.10 In-gel ATPase activity assays 83
6.11 Electron microscopy 83
6.12 Subcellular fractionation 84
Figures 86
Tables 134
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