Stegmüller Judith, Synofzik Matthis
Department of Neurology, University Clinics, RWTH Aachen, Aachen, Germany.
Department of Neurodegenerative Diseases, Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
J Neurochem. 2016 Oct;139(2):159-161. doi: 10.1111/jnc.13775.
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.
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》。