Synphilin-1 transgenic mice exhibit mild motor impairments
Synphilin-1 transgenic mice exhibit mild motor impairments
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DOI：
10.1016/j.neulet.2008.08.073
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发表时间：
2008-11-07
影响因子：
2.5
通讯作者：
Matsumoto, Masayasu
中科院分区：
文献类型：
--
作者：
Jin, Hong-Guo;Yamashita, Hiroshi;Matsumoto, Masayasu
亲突触蛋白-1代表与α-突触核蛋白相互作用并定位于突触囊泡附近的细胞质蛋白。synphilin-1与参与帕金森病的几种蛋白质的相互作用表明，它可能参与疾病的发病机制。尽管如此，synphilin-1的功能仍不清楚。在本研究中，我们产生了转基因小鼠表达人类synphilin-1下朊病毒蛋白启动子。Synphilin-1在包括黑质在内的脑神经元中广泛表达，其中未观察到多巴胺神经元的大量损失。在转基因小鼠脑中，synphilin-1蛋白是多泛素化的，并且部分不溶。虽然修改SHIRPA揭示没有显着差异的行为和形态，减少转基因小鼠的转棒性能和步长观察到与非转基因同窝出生。Synphilin-l可能参与运动功能，其在中枢神经系统中的积累可导致运动障碍。(C)2008爱思唯尔爱尔兰有限公司保留所有权利。
Synphilin-1 represents a cytoplasmic protein that interacts with (x-synuclein and localizes close to synaptic vesicles. The interaction of synphilin-1 with several proteins involved in Parkinson's disease suggests that it might be involved in the pathogenesis of the disease. Nonetheless, the function of synphilin-1 remains unclear. In the present study, we generated transgenic mice expressing human synphilin-1 under the prion protein promoter. Synphilin-1 was widely expressed in neurons in the brain including the substantia nigra, where massive loss of dopamine neurons was not observed. In the transgenic Mouse brain, synphilin-1 protein was polyubiquitinated, and partially insoluble. Although modified-SHIRPA revealed no significant difference in behavior and morphology, the reduced rotarod performance and step length were observed in transgenic mice as compared with non-transgenic littermates. Synphilin-l might be involved in motor function, and its accumulation in the central nervous system can cause motor impairments. (C) 2008 Elsevier Ireland Ltd. All rights reserved.