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大鼠行为学方面的新型转基因肌萎缩侧索硬化症/额颞叶痴呆模型:对《泛素连接酶2表达增加导致转基因大鼠神经元死亡》的编辑亮点

New transgenic ALS/FTD models on the rat-walk: An Editorial Highlight for 'Increased Ubqln2 expression causes neuron death in transgenic rats'.

作者信息

Stegmüller Judith, Synofzik Matthis

机构信息

Department of Neurology, University Clinics, RWTH Aachen, Aachen, Germany.

Department of Neurodegenerative Diseases, Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.

出版信息

J Neurochem. 2016 Oct;139(2):159-161. doi: 10.1111/jnc.13775.

Abstract

This Editorial highlights a study by Huang and colleagues in the current issue of Journal of Neurochemistry. The authors introduce a novel ALS-FTD (amyotrophic lateral sclerosis-frontotemporal dementia) rat model to explore the role of the UBLQN2 gene that has previously been associated with familial ALS-FTD. Over-expression of ubiquilin 2 in the cortex (CTX) and hippocampus of the rat results in ubiquilin 2 aggregates and neurodegeneration together with cognitive deficits. The new rat model not only gives insight into potential molecular underpinnings of ALS-FTD, but also represents an important new tool for future research and therapeutic approaches. Read the highlighted article 'Increased Ubqln2 expression causes neuron death in transgenic rats' on page 285.

摘要

本社论重点介绍了黄及其同事在本期《神经化学杂志》上发表的一项研究。作者引入了一种新型的肌萎缩侧索硬化症-额颞叶痴呆(ALS-FTD)大鼠模型,以探究此前已与家族性ALS-FTD相关的UBLQN2基因的作用。大鼠大脑皮层(CTX)和海马体中泛素连接蛋白2的过度表达会导致泛素连接蛋白2聚集、神经退行性变以及认知缺陷。这种新的大鼠模型不仅有助于深入了解ALS-FTD潜在的分子基础,还代表了未来研究和治疗方法的一种重要新工具。阅读第285页上的重点文章《Ubqln2表达增加导致转基因大鼠神经元死亡》。

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