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利用模式生物解读肌萎缩侧索硬化症的分子逻辑:“家族之事” 。

Deciphering the molecular logic of ALS using model organisms: "A family affair.".

作者信息

Brent Jonathan R, Ozdinler P Hande

机构信息

Department of Neurology and Clinical Neurological Sciences, Northwestern University, Feinberg School of Medicine, Chicago, IL, 60611.

Cognitive Neurology and Alzheimer's disease Center, Northwestern University, Chicago, IL, 60611.

出版信息

SOJ Neurol. 2017;4(1):1-3. doi: 10.15226/2374-6858/4/1/00132. Epub 2017 Jul 19.

Abstract

Recent advances in the genetics of ALS have bolstered hope that a molecular logic for the pathogenesis of the disease is fast approaching. An emerging challenge is the dissection of the common and unique molecular pathways altered by ALS gene mutations. Disease modeling in rodents has yielded many important insights, but as the genetic complexity of the disease grows, additional models with improved speed, cost and genetic tractability will be increasingly necessary. Models such as fruitfly, nematode, and zebrafish have been important for diagramming the molecular pathways that underlie many fundamental biological processes, but have been comparatively underutilized in the study of neurodegeneration. Here we highlight the benefits and opportunities for increased diversity in the models used to study ALS.

摘要

肌萎缩侧索硬化症(ALS)遗传学的最新进展增强了人们的希望,即该疾病发病机制的分子逻辑即将明晰。一个新出现的挑战是剖析由ALS基因突变改变的共同和独特分子途径。在啮齿动物中进行疾病建模已经产生了许多重要见解,但随着该疾病遗传复杂性的增加,具有更高速度、更低成本和更好遗传易处理性的其他模型将变得越来越必要。果蝇、线虫和斑马鱼等模型对于描绘许多基本生物学过程背后的分子途径很重要,但在神经退行性疾病研究中相对未得到充分利用。在这里,我们强调了用于研究ALS的模型增加多样性的好处和机会。

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