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完整後設資料紀錄
DC 欄位 | 值 | 語言 |
---|---|---|
dc.contributor.advisor | 湯志永(Chih-Yung Tang) | |
dc.contributor.author | Jing-Jia Huang | en |
dc.contributor.author | 黃敬家 | zh_TW |
dc.date.accessioned | 2021-06-08T00:51:41Z | - |
dc.date.copyright | 2015-09-25 | |
dc.date.issued | 2015 | |
dc.date.submitted | 2015-06-29 | |
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dc.identifier.uri | http://tdr.lib.ntu.edu.tw/jspui/handle/123456789/18116 | - |
dc.description.abstract | 正常的骨骼肌細胞膜藉由表達大量的氯離子通道而維持細胞膜電位的穩定。過去的文獻報告指出,當骨骼肌細胞中第7對染色體上的CLCN1基因發生變異,可能會導致膜上電位依賴性氯離子通道CLC-1功能異常,繼而引發先天性肌強直症(myotonia congenita)的產生。本實驗室先前的研究發現,與肌強直症相關的CLC-1突變型A531V雖然在功能及閘道調控機制上與野生型(wild-type)並無明顯差異,但生成的CLC-1 A531V無法順利地由ER運送至細胞膜上表現,且由於蛋白酶體降解 (proteasome degradation) 增加導致蛋白質的總表現量減少,並因此造成細胞膜上的氯離子流密度 (Cl- current density) 明顯下降。若利用dominant negative (DN)-CUL4A/4B干擾CLC-1泛素蛋白酶體降解路徑中CRL4 E3 ligase complex的構築,或是藉由Ub-K0阻礙泛素鏈的聚合,則會促使細胞質中CLC-1的總表現量增加,顯示CLC-1可能是經由CRL4媒介泛素連結以及蛋白酶體降解路徑。此外,當Hsc70、FKBP8、Aha1及HOP大量表現後,亦會造成細胞質中CLC-1的總表現量增加,所以新生成的CLC-1可能藉由Hsp70-Hsp90伴護蛋白系統調控蛋白分子的折疊及穩定。然而,藉由干擾蛋白質降解路徑或促進Hsp70-Hsp90伴護蛋白系統所增加的CLC-1蛋白質並不一定能夠順利地被運送至細胞膜成為具有功能性的氯離子通道。因此,本論文以電生理的角度,探討影響CLC-1之Hsp70-Hsp90伴護蛋白系統及蛋白酶體降解途的參與因子,是否的確會影響細胞膜上CLC-1氯離子通道蛋白的功能表達。我們的實驗結果顯示,若增加Hsp70-Hsp90伴護蛋白系統中參與因子Aha1、FKBP8、HOP、Hsp90α/β及Hsc/Hsp70的表現量,得以促進CLC-1野生型和A531V氯離子通道的功能表現。此外,利用MLN4924藥物處理細胞,藉由DN-CUL4A/4B及DN-CRBN干擾CRL4的構築,或利用Ub-K0阻礙泛素鏈的聚合,亦皆能有效增加細胞膜上CLC-1通道之氯離子流。綜觀我們的研究結果足以推測,Hsp70-Hsp90伴護蛋白系統與CRL4蛋白酶體降解系統的確可以經由影響CLC-1蛋白質生成而調控細胞膜上的CLC-1氯離子通道之功能表現。 | zh_TW |
dc.description.abstract | The expression of chloride channels is essential for the stabilization of membrane potential in skeletal muscle. Myotonia congenita is a skeletal muscle disorder associated with mutations in the CLCN1 gene (chromosome 7q), which encodes the voltage-gated chloride channel, CLC-1. Previous studies from our lab have demonstrated that despite the presence of a diminished whole-cell current density, the myotonia-related CLC-1 mutant A531V displays no detectable gating defects. Yet the A531V mutant is associated to a defective endoplasmic reticulum export and a reduction in the total protein expression that can be attributed to enhanced proteasomal degradation. Co-expression with Ub-K0 or dominant negative (DN)-CUL4A/4B increased the protein expression level of CLC-1, suggesting that the CRL4 E3 ligase system may mediate the ubiquitination and thus the proteasome degradation of CLC-1 proteins. Furthermore, over-expression of Hsc70, FKBP8, Aha1, or HOP also promoted CLC-1 expression, implying that the Hsp70-Hsp90 system may contribute to CLC-1 protein folding and stability. Elevated expression level of CLC-1 proteins, however, does not prove that the recued CLC-1 proteins are trafficked to cell surface and display proper chloride channel function. Hence, in this study, we aim to apply electrophysiological techniques to determine whether the Hsp70-Hsp90 and the CRL4 proteasome degradation systems modulate functional CLC-1 expression. Our results show that Aha1, FKBP8, HOP, Hsp90α/β, or Hsc/Hsp70 enhanced the functional expression WT and A531V CLC-1 channels. CLC-1 currents were also increased upon suppression of CRL4 function (by MLN4924 or DN-CUL4A/4B) or ubiquitin chain formation (by Ub-K0). Together, our data suggest that the Hsp70-Hsp90 system and the CRL4 proteasome degradation systems can indeed modulate the functional expression CLC-1 channels through the regulation of the biosynthesis of the channel protein. | en |
dc.description.provenance | Made available in DSpace on 2021-06-08T00:51:41Z (GMT). No. of bitstreams: 1 ntu-104-R02441001-1.pdf: 6170938 bytes, checksum: dec033b61b6d9cb8ba129d8af0e07c3d (MD5) Previous issue date: 2015 | en |
dc.description.tableofcontents | 中文摘要 i Abstract iii 圖目錄 viii 第一章 導論 1 1.1.1 氯離子通道 (Chloride channels) 1 1.1.2 CLC-1所扮演的功能及生理意義 2 1.1.3 CLC-1的結構與開關調控 3 1.1.4 先天性肌強直症(Myotonia congenita) 4 1.1.5 Myotonia mutant: A531V 6 1.2.1 分子伴護蛋白調控細胞內蛋白質的平衡 (proteostasis) 7 1.2.2 分子伴護蛋白協助細胞內蛋白質的摺疊及結構維持 9 1.2.3 分子伴護蛋白參與細胞內蛋白質的降解 11 1.2.4 The Hsp70 System 13 1.2.5 The Hsp90 System 15 1.2.6 The Chaperonin System 17 1.2.7 細胞內蛋白質降解系統 18 1.2.8 溶酶體降解 (lysosomal proteolysis) 18 1.2.9 泛素蛋白酶體系統 (ubiquitin-proteasome system) 19 1.2.10 Membrane trafficking 21 1.3 研究目的 23 第二章 材料與方法 25 2.1 DNA construct 25 2.2 Cell culture 25 2.3 DNA transfection 26 2.4 藥物處理細胞 26 2.5 Electrophysiological recordings 27 2.6 統計分析 29 第三章 結果 30 3.1 Cochaperones Aha1及FKBP8提升macroscopic Cl- current 30 3.2 Cochaperone HOP提升macroscopic Cl- current 31 3.3 Chaperone Hsp90增加macroscopic Cl- current 32 3.4 Chaperone Hsp70增加macroscopic Cl- current 33 3.5 17-AAG增加macroscopic Cl- current 34 3.6 MLN4924增加macroscopic Cl- current 34 3.7 大量表現CUL4A/B、DDB1或CRBN對macroscopic Cl- current有不同的影響 35 3.8 DN-CUL4與DN-CRBN增加macroscopic Cl- current 36 3.9 Thalidomide藥物處理不影響macroscopic Cl- current 38 3.10 Znrf1 E3 ligase大量表現不影響macroscopic Cl- current 38 3.11 干擾CLC-1之polyubiquitin chain形成增加macroscopic Cl- current 39 第四章 討論 41 4.1 Aha1調控Hsp90 cycling影響細胞膜上功能性CLC-1表現 41 4.2 FKBP8促進細胞膜上功能性CLC-1氯離子通道表現 42 4.3 Hsp70影響細胞膜上CLC-1功能性表現 44 4.4 Hsp90影響細胞膜上CLC-1功能性表現 46 4.5 17-AAG對於CLC-1之影響 48 4.6 CRL4 in CLC-1 ubiquitin-proteasome degradation pathway 48 4.7 Thalidomide藥物處理對於CLC-1之影響 50 4.8 Znrf1 E3 ligase對於macroscopic Cl- current之影響 51 4.9 ATP造成CLC-1通道G-V curve偏移 51 4.10 Voltage errors in large current amplitude recordings 52 4.11 CLC-1折疊及降解路徑之可能模式 54 4.12 待釐清之問題及未來的實驗方向 55 結論 58 圖表 59 附圖 91 附圖一、 Schematic representation of the ClC-1 channel 91 附圖二、 CLC-1 single-channel events 92 附圖三、 真核細胞內的伴護蛋白基本調控途徑 93 附圖四、 Model of polytopic membrane protein biogenesis 94 參考資料 95 | |
dc.language.iso | zh-TW | |
dc.title | 分子伴護蛋白及泛素連接酶調控人類第一型氯離子通道之功能表現 | zh_TW |
dc.title | Modulation of CLC-1 functional expression by molecular chaperones and ubiquitin ligases | en |
dc.type | Thesis | |
dc.date.schoolyear | 103-2 | |
dc.description.degree | 碩士 | |
dc.contributor.oralexamcommittee | 鄭瓊娟,胡孟君,吳君泰 | |
dc.subject.keyword | 分子伴護蛋白,泛素連接?,人類第一型氯離子通道, | zh_TW |
dc.subject.keyword | molecular chaperone,ubiquitin ligase, | en |
dc.relation.page | 107 | |
dc.rights.note | 未授權 | |
dc.date.accepted | 2015-06-29 | |
dc.contributor.author-college | 醫學院 | zh_TW |
dc.contributor.author-dept | 生理學研究所 | zh_TW |
顯示於系所單位: | 生理學科所 |
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