A fruitful endeavor: modeling ALS in the fruit fly.

A fruitful endeavor: modeling ALS in the fruit fly.
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DOI:
10.1016/j.brainres.2014.09.064
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发表时间:
2015-05-14
期刊:
影响因子:
2.9
通讯作者:
Pandey, Udai Shan
Pandey, Udai Shan
中科院分区:
医学3区
文献类型:
--
作者:
Casci, Ian;Pandey, Udai Shan

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世纪以来,黑腹果蝇(Drosophila melanogaster),俗称果蝇,一直在遗传学研究和疾病建模方面发挥着重要作用。近年来,它已成为建模和研究神经退行性疾病的有力工具,包括毁灭性和致命的肌萎缩侧索硬化症(ALS)。这种模式生物在ALS研究中的成功来自于操纵许多所需细胞类型中基因/蛋白质表达的工具的可用性,以及随后对该疾病的细胞和分子表型特征的重演。现在已经开发了几种果蝇模型来研究ALS相关基因在疾病发病机制中的作用,这使我们能够了解导致ALS患者运动神经元变性的分子途径。我们在这篇综述中的主要目标是强调我们使用果蝇模型与ALS研究有关的经验教训。
For over a century Drosophila melanogaster, commonly known as the fruit fly, has been instrumental in genetics research and disease modeling. In more recent years, it has been a powerful tool for modeling and studying neurodegenerative diseases, including the devastating and fatal amyotrophic lateral sclerosis (ALS). The success of this model organism in ALS research comes from the availability of tools to manipulate gene/protein expression in a number of desired cell-types, and the subsequent recapitulation of cellular and molecular phenotypic features of the disease. Several Drosophila models have now been developed for studying the roles of ALS-associated genes in disease pathogenesis that allowed us to understand the molecular pathways that lead to motor neuron degeneration in ALS patients. Our primary goal in this review is to highlight the lessons we have learned using Drosophila models pertaining to ALS research.
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