王桂馨博士
長聘副研究員
- 2789-9051 (Lab) (Room No: 206)
- 2652-3051 (Office)
- 2782-3047 (Fax)
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Specialty:
- Cardiovascular disease
- Neurodegeneration
- Neurodevelopmental disorder
Education and Positions:
Ph.D. National Yang-Ming University
本實驗室的研究有兩個主題：(1) 探究造成神經發育遲緩的致病機轉; (2) 研究造成神經退化疾病的致病機轉。我們以一顯性罕見遺傳疾病：「肌強直症」的小鼠模式，作為主要研究的工具。肌強直症為一顯性遺傳疾病，患者具有肌肉僵直、心血管系統及神經系統方面之疾病。造成疾病的主要原因是由於，數百或上千之CTG repeat 位在DMPK 基因非轉譯區，經轉錄成RNA之後形成穩定之二級結構，此RNA累積在細胞核內影響細胞之生理功能。
(1)「肌強直症」的患者，若神經系統發育過程受阻所造成之症狀包括，心智發展遲緩、過動及缺乏注意力集中、嗜睡、學習記憶障礙及精神方面等疾病。罹患肌強直症的病人其認知功能異常與行為偏差的比例很高，然而，目前對於造成神經系統方面的疾病之原因仍不清楚。我們希望透過研究其中的致病機轉，進而瞭解如何找到減緩疾病症狀的治療方法。
(2) 不同的神經退化性疾病，雖然致病的基因各有不同，包括阿茲海默症及「肌強直症」，卻具有共同的退化表徵，例如記憶及行動力退化，代謝異常。致病機轉。我們過去的研究結果提出一個神經退化疾病晚期共通的分子機轉。我們希望以「肌強直症」為研究的工具，找出神經退化的歷程中重要的表徵，以及造成退化的早期之分子機轉。
我們期望透過研究「肌強直症」在神經系統的致病機轉，可以進而瞭解廣泛的神經發育及神經退化形成的原因。
Our Team
- Wang PY, Kuo TY, Wang LH, Liang WS, Wang, GS Loss of MBNL1 mediated retrograde BDNF signaling in myotonic dystrophy brain. ACTA NEUROPATHOLOGICA COMMUNICATIONS 11, 44-56 (2023-03-15) [JCR] [WOS]
- Lee-Hsin Wang , Chien-Yu Lin, Yu-Mei Lin, Luc Buée, Nicolas Sergeant, David Blum, Yijuang Chern , Guey-Shin Wang Calpain-2 Mediates MBNL2 Degradation and a Developmental RNA Processing Program in Neurodegeneration JOURNAL OF NEUROSCIENCE 42, 5102-5114 (2022-06-22) [JCR] [WOS]
- Chang KT, Wang LH, Lin YM, Cheng CF, Wang GS. CELF1 promotes vascular endothelial growth factor degradation resulting in impaired microvasculature in heart failure FASEB JOURNAL 35(5), e21512 (2021-05) [JCR] [WOS]
- Wang PY, Chang KT, Lin YM, Kuo TY, Wang GS* Ubiquitination of MBNL1 Is Required for Its Cytoplasmic Localization and Function in Promoting Neurite Outgrowth CELL REPORTS 22, 2294-2306 (2018-02) [JCR] [WOS]
- Chang KT, Cheng CF, King PC, Liu SY, Wang GS* CELF1 Mediates Connexin 43 mRNA Degradation in Dilated Cardiomyopathy. CIRCULATION RESEARCH 121, 1140-1152 (2017) [JCR] [WOS]
- Wang PY, Lin YM, Wang LH, Kuo TY, Cheng SJ, Wang GS* Reduced cytoplasmic MBNL1 is an early event in a brain-specific mouse model of myotonic dystrophy. HUMAN MOLECULAR GENETICS 26(12), 2247-2257 (2017) [JCR] [WOS]
- Verma SK, Deshmukh V, Liu P, Nutter CA, Espejo R, Hung ML, Wang GS, Yeo GW, Kuyumcu-Martinez MN. Reactivation of fetal splicing programs in diabetic hearts is mediated by protein kinase C signaling. J Biol Chem. 288(49), 35372-35386 (2013-12) [JCR] [WOS]
- Wang GS, Kuyumcu-Martinez MN, Sarma S, Mathur N, Wehrens XH, Cooper TA PKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1. JOURNAL OF CLINICAL INVESTIGATION 119(12), 3797-3806 (2009-12) [JCR] [WOS]
- Wang GS, Kearney DL, De Biasi M, Taffet G, Cooper TA Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy. JOURNAL OF CLINICAL INVESTIGATION 117(10), 2802-2811 (2007) [JCR] [WOS]
- Kuyumcu-Martinez NM, Wang GS, Cooper TA Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation. MOLECULAR CELL 28(1), 68-78 (2007) [JCR] [WOS]
POSTDOC
張貴婷
王李馨
RESEARCH ASSOCIATES
梁文馨
余湘羚
ALUMNI
洪子恆
邱瑀絜
蘇芷嫻
汪佩瑩
李婉萱
- 誠徵: 博士後研究 1名