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Genetically engineered animal models of Parkinson's disease: From worm to rodent

机译:帕金森病的基因工程动物模型:从蠕虫到啮齿动物

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Abstract Parkinson's disease ( PD ) is a progressive neurological disorder characterised by aberrant accumulation of insoluble proteins, including alpha‐synuclein, and a loss of dopaminergic neurons in the substantia nigra. The extended neurodegeneration leads to a drop of striatal dopamine levels responsible for disabling motor and non‐motor impairments. Although the causes of the disease remain unclear, it is well accepted among the scientific community that the disorder may also have a genetic component. For that reason, the number of genetically engineered animal models has greatly increased over the past two decades, ranging from invertebrates to more complex organisms such as mice and rats. This trend is growing as new genetic variants associated with the disease are discovered. The EU Joint Programme – Neurodegenerative Disease Research ( JPND ) has promoted the creation of an online database aiming at summarising the different features of experimental models of Parkinson's disease. This review discusses available genetic models of PD and the extent to which they adequately mirror the human pathology and reflects on future development and uses of genetically engineered experimental models for the study of PD .
机译:摘要帕金森病(PD)是一种渐进式神经障碍,其特征是不溶性蛋白质的异常积累,包括α-突触核蛋白,以及在体积NIGRA中的多巴胺能神经元的丧失。延长的神经变性导致侧面的纹状体多巴胺水平,负责禁用电机和非运动损伤。虽然疾病的原因仍然尚不清楚,但在科学群落中,这种疾病也可能具有遗传组分。因此,在过去二十年中,转基因动物模型的数量大大增加,从无脊椎动物到更复杂的生物,如小鼠和大鼠。这种趋势正在增长,因为发现与疾病相关的新遗传变异。欧盟联合计划 - 神经退行性疾病研究(JPND)促进了一个在线数据库的创建,旨在总结帕金森病的实验模型的不同特征。该评论讨论了PD的可用遗传模型以及它们充分镜像人类病理的程度,并反映了未来的开发和基因工程实验模型的应用研究。

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