https://scholars.lib.ntu.edu.tw/handle/123456789/588046
標題: | Restoring Bcl-xL levels benefits a mouse model of spinal muscular atrophy | 作者: | LI-KAI TSAI Tsai M.-S. Ting C.-H. Wang S.-H. Li H. |
公開日期: | 2008 | 卷: | 31 | 期: | 3 | 起(迄)頁: | 361-367 | 來源出版物: | Neurobiology of Disease | 摘要: | Currently, no curative treatment is available for spinal muscular atrophy (SMA). Since the degeneration of spinal motor neurons in SMA is mediated by apoptosis, over-expression of an anti-apoptotic factor, Bcl-xL, may benefit SMA. Here, we crossed a mouse model of SMA with Bcl-xL transgenic mice to create SMA/Bcl-xL mice. The Bcl-xL expression in the spinal neurons of SMA/Bcl-xL mice was nearly double that in SMA mice. SMA/Bcl-xL mice showed preserved motor function, normalized electrophysiological tests, diminished muscle atrophy, and less motor neuron degeneration. In addition, the life span of SMA/Bcl-xL mice was 1.5 times longer than that of SMA mice. Therefore, over-expression of Bcl-xL has a potential for amelioration of SMA, and Bcl-xL may be another attractive therapeutic target other than survival motor neuron (SMN) protein for use in future drug screening for SMA. ? 2008 Elsevier Inc. All rights reserved. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-49349107825&doi=10.1016%2fj.nbd.2008.05.014&partnerID=40&md5=74bbdffd211fb27296a89af31582beb3 https://scholars.lib.ntu.edu.tw/handle/123456789/588046 |
ISSN: | 9699961 | DOI: | 10.1016/j.nbd.2008.05.014 | SDG/關鍵字: | protein bcl xl; animal cell; animal experiment; animal model; animal tissue; article; cell structure; controlled study; electrophysiology; lifespan; motor performance; mouse; nonhuman; priority journal; protein expression; spinal cord nerve cell; spinal muscular atrophy; transgenic mouse; Animals; Apoptosis; bcl-X Protein; Cell Survival; Crosses, Genetic; Disease Models, Animal; Drug Design; Drug Evaluation, Preclinical; Gene Therapy; Humans; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Motor Neurons; Muscular Atrophy; Muscular Atrophy, Spinal; Nerve Degeneration; Spinal Cord; Survival Rate |
顯示於: | 醫學院附設醫院 (臺大醫院) |
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