Molecular insights into cortico-striatal miscommunications in Huntington's disease.
Molecular insights into cortico-striatal miscommunications in Huntington's disease.
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DOI：
10.1016/j.conb.2017.10.019
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发表时间：
2018-03
影响因子：
5.7
通讯作者：
Yang XW
中科院分区：
文献类型：
--
作者：
Veldman MB;Yang XW
亨廷顿氏病（HD）是一种显性遗传性神经退行性疾病，由其遗传原因、HTT基因中的CAG重复扩增、其运动和精神病学以及纹状体中型棘状神经元（MSN）的原发性损失来定义。然而，从遗传损伤到疾病表型的分子机制仍不清楚。已经建立了HD小鼠模型，其表现出与患者相似的表型，具体而言，皮质-纹状体连接断开似乎是HD小鼠模型和患者共有的共同致病事件。分子研究已经开始揭示一致的分子和细胞致病机制，可能解释各种HD小鼠模型中皮质-纹状体的错误通讯。系统生物学方法有助于阐明突触分子网络作为HD皮质-纹状体发病机制的联系，并可能提供新的候选靶点来改变疾病。
Huntington’s disease (HD), a dominantly inherited neurodegenerative disease, is defined by its genetic cause, a CAG-repeat expansion in the HTT gene, its motor and psychiatric symptomology and primary loss of striatal medium spiny neurons (MSNs). However, the molecular mechanisms from genetic lesion to disease phenotype remain largely unclear. Mouse models of HD have been created that exhibit phenotypes reminiscent of those in the patient, and specifically, cortico-striatal disconnectivity appears to be a shared pathogenic event shared by HD mouse models and patients. Molecular studies have begun to unveil consistent molecular and cellular pathogenic mechanisms that may account for cortico-striatal miscommunication in various HD mouse models. Systems biological approaches help to illuminate synaptic molecular networks as a nexus for HD cortio-striatal pathogenesis, and may offer new candidate targets to modify the disease.
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影响因子：
3
作者：
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通讯作者：
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