Lu Bingwei
Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA.
Apoptosis. 2009 Aug;14(8):1008-20. doi: 10.1007/s10495-009-0347-5.
Neurodegenerative diseases are progressive disorders of the nervous system that affect the function and maintenance of specific neuronal populations. Most disease cases are sporadic with no known cause. The identification of genes associated with familial cases of these diseases has enabled the development of animal models to study disease mechanisms. The model organism Drosophila has been successfully used to study pathogenic mechanisms of a wide range of neurodegenerative diseases. Recent genetic studies in the Drosophila models have provided new insights into disease mechanisms, emphasizing the roles played by mitochondrial dynamics, RNA (including miRNA) function, protein translation, and synaptic plasticity and differentiation. It is anticipated that Drosophila models will further our understanding of mechanisms of neurodegeneration and facilitate the development of novel and rational treatments for these debilitating neurodegenerative diseases.
神经退行性疾病是神经系统的进行性疾病,会影响特定神经元群体的功能和维持。大多数病例是散发性的,病因不明。与这些疾病的家族性病例相关的基因的鉴定,使得能够开发动物模型来研究疾病机制。模式生物果蝇已成功用于研究多种神经退行性疾病的致病机制。最近在果蝇模型中的遗传学研究为疾病机制提供了新的见解,强调了线粒体动力学、RNA(包括miRNA)功能、蛋白质翻译以及突触可塑性和分化所起的作用。预计果蝇模型将加深我们对神经退行性变机制的理解,并促进针对这些使人衰弱的神经退行性疾病的新型合理治疗方法的开发。