Drosophila as an In Vivo Model for Human Neurodegenerative Disease
Drosophila as an In Vivo Model for Human Neurodegenerative Disease
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DOI：
10.1534/genetics.115.179457
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发表时间：
2015-10-01
期刊：
影响因子：
3.3
通讯作者：
Bonini, Nancy M.
中科院分区：
文献类型：
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作者：
McGurk, Leeanne;Berson, Amit;Bonini, Nancy M.
随着人口老龄化的加剧，神经退行性疾病给家庭带来毁灭性打击，给社会带来巨大负担。大脑和脊髓非常复杂：它们由高度组织的神经元和支持细胞网络组成，这些细胞以高度专业化的方式进行通信。解决如此复杂问题的一种方法是在更简单但类似的系统中解决科学问题。果蝇（Drosophila melanogaster）已被证明作为一种模式生物具有巨大的价值，它使神经科学疾病研究中的许多重大发现成为可能。果蝇中有大量可用的遗传工具，可以对基因组进行精确的有针对性的操作。由于其寿命相对较短，因此与其他模型生物（例如小鼠）相比，可以更快地解决大脑功能的复杂问题。在这里，我们讨论苍蝇作为人类神经退行性疾病模型的特征。对于一系列神经退行性疾病，有许多不同的果蝇模型；我们重点关注多聚谷氨酰胺疾病和肌萎缩侧索硬化症模型的精选研究，这些研究说明了可以收集的见解的类型和范围。在讨论这些模型时，我们强调了果蝇在提供对机制和途径的理解方面的优势，作为转化和治疗研究的基础。
With the increase in the ageing population, neurodegenerative disease is devastating to families and poses a huge burden on society. The brain and spinal cord are extraordinarily complex: they consist of a highly organized network of neuronal and support cells that communicate in a highly specialized manner. One approach to tackling problems of such complexity is to address the scientific questions in simpler, yet analogous, systems. The fruit fly, Drosophila melanogaster, has been proven tremendously valuable as a model organism, enabling many major discoveries in neuroscientific disease research. The plethora of genetic tools available in Drosophila allows for exquisite targeted manipulation of the genome. Due to its relatively short lifespan, complex questions of brain function can be addressed more rapidly than in other model organisms, such as the mouse. Here we discuss features of the fly as a model for human neurodegenerative disease. There are many distinct fly models for a range of neurodegenerative diseases; we focus on select studies from models of polyglutamine disease and amyotrophic lateral sclerosis that illustrate the type and range of insights that can be gleaned. In discussion of these models, we underscore strengths of the fly in providing understanding into mechanisms and pathways, as a foundation for translational and therapeutic research.