Chaperoning brain degeneration
Chaperoning brain degeneration
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DOI：
10.1073/pnas.152330499
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发表时间：
2002-12-10
影响因子：
11.1
通讯作者：
Bonini, NM
中科院分区：
文献类型：
--
作者：
Bonini, NM
果蝇已经成为研究人类神经退行性疾病的首要模型系统。与神经变性相关的基因可以在果蝇中表达，导致与人类疾病的表型非常相似的表型。由于人类神经退行性疾病，包括亨廷顿氏病和帕金森氏病，是几乎没有治愈或治疗方法的疾病，果蝇为这些疾病的问题带来了强大的遗传学。分子伴侣是第一个定义的修饰剂，在果蝇中干扰这种疾病表型的进展。热休克蛋白70是果蝇多聚谷氨酰胺病和帕金森病的有效抑制因子。这些研究提供了通过上调应激和分子伴侣途径治疗人类神经退行性疾病的希望。
Drosophila has emerged as a premiere model system for the study of human neurodegenerative disease. Genes associated with neurodegeneration can be expressed in flies, causing phenotypes remarkably similar to those of the counterpart human diseases. Because human neurodegenerative diseases, including Huntington's and Parkinson's diseases, are disorders for which few cures or treatments are available, Drosophila brings to bear powerful genetics to the problem of these diseases. The molecular chaperones were the first modifiers defined that interfere in the progression of such disease phenotypes in Drosophila. Hsp70 is a potent suppressor of both polyglutamine disease and Parkinson's disease in Drosophila. These studies provide the promise of treatments for human neurodegeneration through the up-regulation of stress and chaperone pathways.