https://scholars.lib.ntu.edu.tw/handle/123456789/526005
標題: | Establishing a standardized therapeutic testing protocol for spinal muscular atrophy | 作者: | LI-KAI TSAI Tsai M.-S. Lin T.-B. WUH-LIANG HWU Li H. |
公開日期: | 2006 | 卷: | 24 | 期: | 2 | 起(迄)頁: | 286-295 | 來源出版物: | Neurobiology of Disease | 摘要: | Several mice models have been created for spinal muscular atrophy (SMA); however, there is still no standard preclinical testing system for the disease. We previously generated type III-specific SMA model mice, which might be suitable for use as a preclinical therapeutic testing system for SMA. To establish such a system and test its applicability, we first created a testing protocol and then applied it as a means to investigate the use of valproic acid (VPA) as a possible treatment for SMA. These SMA mice revealed tail/ear/foot deformity, muscle atrophy, poorer motor performances, smaller compound muscle action potential and lower spinal motoneuron density at the age of 9 to 12?months in comparison with age-matched wild-type littermate mice. In addition, VPA attenuates motoneuron death, increases spinal SMN protein level and partially normalizes motor function in SMA mice. These results suggest that the testing protocol developed here is well suited for use as a standardized preclinical therapeutic testing system for SMA. ? 2006 Elsevier Inc. All rights reserved. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-33750001213&doi=10.1016%2fj.nbd.2006.07.004&partnerID=40&md5=73aee88244b41f40beb68aba4867f195 https://scholars.lib.ntu.edu.tw/handle/123456789/526005 |
ISSN: | 0969-9961 | DOI: | 10.1016/j.nbd.2006.07.004 | SDG/關鍵字: | p 4543; survival motor neuron protein; valproic acid; animal experiment; animal model; article; cell density; comparative study; controlled study; ear malformation; foot malformation; motoneuron; motor performance; mouse; muscle action potential; muscle atrophy; nerve cell necrosis; nonhuman; priority journal; spinal muscular atrophy; wild type; Animals; Cell Death; Cell Survival; Cyclic AMP Response Element-Binding Protein; Disease Models, Animal; Drug Evaluation, Preclinical; Enzyme Inhibitors; Female; Histone Deacetylase Inhibitors; Histone Deacetylases; Humans; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Motor Neurons; Muscle, Skeletal; Muscular Atrophy, Spinal; Nerve Degeneration; Nerve Tissue Proteins; Neuroprotective Agents; Predictive Value of Tests; RNA-Binding Proteins; SMN Complex Proteins; Spinal Cord; Treatment Outcome; Valproic Acid |
顯示於: | 醫學系 |
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