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等级： 7级谢氏网贵宾
贴子：25159
积分：60554
谢氏币：41
来自：重庆
注册：2006-04-23 |
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第 1987 楼 |
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 谢明丽 东海生命科学研究中心生命科学系教授 |
谢明丽，女，东海生命科学研究中心生命科学系教授，自台湾大学化学系毕业，并于美国加州大学洛杉矶分校获得生物化学博士学位，研究领域是转录调控机制、遗传性疾病的研究。主要利用生化分析、基因体学及蛋白质体学专研小脑脊髓运动失调症分子诊断与治病之机转。
研究方向着重于体染色体显性的遗传性小脑脊髓运动失调症(SCAs) 的研究，属于渐进性晚发型神经退化性疾病的亚型。已知调控SCA症状的基因分别座落于不同的染色体，相关基因已选殖完成;在这些基因的转译区(translated region)内均有一段CAG核酸重复序列发生倍增突变是造成此类疾病的原因。陆续搜集了八十多个体染色体显性遗传小脑脊髓运动失调症家族，目前已建立完整检验SCAs的方法，对于台湾地区体染色体显性遗传小脑脊髓运动失调症病人作不同基因型的筛检，目前已在国内找到了患有MJD疾病的约二十六个家族，九个SCA2的家庭，SCA1疾病的一个家族，及一个SCA7 家族。本实验室也研究发展快速检验的方法以对于此类疾病的筛检及追踪调查。目前已能利用绒毛细胞或羊水细胞的DNA分析相关基因中的CAG扩张重复序列，并且实际应用于SCA3/MJD及 SCA2疾病的产前诊断，以期达到优生保健的目的。建立不同MJD/SCA3及 SCA7不同发病年龄患者、未发病者及正常控制组的淋巴母细胞株，更进一步已建立稳定表达SCA3/MJD致病蛋白的人类神经细胞株(SK-N-SH-MJD78)，并制得SCA3/MJD荧光融合蛋白，预计以蛋白体学二维胶体电泳及微矩阵芯片等方法研究，能够更清楚了解不同药物对小脑脊髓运动失调疾病的治疗所扮演的角色，环境因子的影响及此类晚发型疾病致病机转的研究。
期刊论文
1. Chang WH , Cemal CK, Hsu YH, Kuo CL, Nukina N, Chang MH, Hu HT , Li C, Hsieh M* Dynamic expression of Hsp27 in the presence of mutant ataxin-3. Biochem Biophys Res Commun. 2005, 336, 258-267 (SCI)
2. Tsai HF, Lin SJ, Li C, Hsieh M* Decreased expression of Hsp27 and Hsp70 in transformed lymphoblastoid cells from patients with spinocerebellar ataxia type 7. Biochem Biophys Res Commun. 2005, 334, 1279-1286 (SCI)
3. Wu TT, Tsai TW, Chu CT, Lee ZF, Hung CM, Su CC, Li SY, Hsieh M, Li C. Low RET mutation frequency and polymorphism analysis of the RET and EDNRB genes in patients with Hirschsprung disease in Taiwan. J Hum Genet. 2005, 50, 168-174. (SCI)
4. Liu CS , Hsu HM, Cheng WL, Hsieh M*. Clinical and Molecular Events in Patients with Machado-Joseph Disease under Lamotrigine Therapy. Acta Neurol. Scand. 2005, 111, 385-390. (SCI)
5.Hsieh M*, Tsai HF, Chang WH. HSP27 and cell death in Spinocerebellar Ataxia Type 3. The Cerebellum 2005, 4, 31-36.
6. Chen, D-H, Wu, KT, Hung, CJ, Hsieh M, and Li C. Effects of Adenosine
Dialdehyde Treatment on in vitro and in vivo Stable Protein Methylation in
HeLa Cells. J. Biochem.2004, 136, 371-376.(SCI)
7. Tsai HF, Tsai HJ, Hsieh M* Full-Length Expanded Ataxin-3 Enhances Mitochondrial-Mediated Cell Death and Decreases Bcl-2 Expression in Human Neuroblastoma Cells. Biochem. Biophys. Res. Commun. 2004, 324, 1274–1282. (SCI)
8. Liu CS, Hsieh M, Chen YY, Lo MC. Machado-Joseph Shoe. Movement Disorder 2004, 20, 66-68.(SCI)
9. Tsai HF, Liu CS, Leu TM, Wen FC, Lin SJ, Liu CC, Yang DK, Li C, and Hsieh M*. Analysis of trinucleotide repeats in different SCA loci in spinocerebellar ataxia patients and in normal population of Taiwan. Acta Neurol. Scand. 2004, 109, 355-60. (SCI)
10. Wen FC, Li YH, Tsai HF, Lin CH, Li C, Liu SS, Lii CK, Nukina N, and Hsieh M*. Down-Regulation of Heat Shock Protein 27 in Neuronal Cells and Non-neuronal cells Expressing Mutant Ataxin-3. FEBS Letters 2003, 546, 307-314. (SCI)
11. Tsai HF, Liu CS, Chen GD, Lin ML, Li C, Chen YY, Wang BT and Hsieh M*. Prenatal diagnosis of Machado-Joseph disease/ Spinocerebellar Ataxia Type 3 in Taiwan: early detection of expanded ataxin-3. Journal of Clinical Laboratory Analysis 2003, 17, 195-200. (SCI)
12. Lin CH, Huang HM, Hsieh M, Pollard KM, Li C. Arginine methylation of recombinant murine fibrillarin by protein arginine methyltransferase. J Protein Chem 2002, 21, 447-53. (SCI)
13. Huang H-M, Tam MF, Tam T-C, Chen D-H, Hsieh M, and Li C. Proteomic Analysis of stable Protein Methylation in Lymphoblastoid Cells. J Biochem (Tokyo) 2002, 132, 813-818. (SCI)
14. Gaspar C, Lopes-Cendes I, Hayes S, Goto J, Arvidsson K, Dias A, Silveira I, Maciel P, Coutinho P, Lima M, Zhou YX, Soong BW, Watanabe M, Giunti P, Stevanin G, Riess O, Sasaki H, Hsieh M, Nicholson GA, Brunt E, Higgins JJ, Lauritzen M, Tranebjaerg L, Volpini V, Wood N, Ranum L, Tsuji S, Brice A, Sequeiros J and Rouleau GA. Ancestral origins of the Machado-Joseph disease mutation: a worldwide haplotype study. Am J Hum Genet 2001, 68, 523-8. (SCI)
15. Liu CS, Sun MH, Kuo CL, Tsai CS, Lu HC, Tsai CF, Tsai CF and Hsieh M Molecular, Clinical Studies and Review in Spinocerebellar Ataxia Type 2 The Changhua Journal of Medicine 2001, 6, 107-112.
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