内质网应激再次作祟：肌萎缩侧索硬化症中的蛋白质稳态功能障碍
ER strikes again: Proteostasis Dysfunction In ALS.
作者信息
Maharjan Niran, Saxena Smita
机构信息
Institute of Cell Biology, University of Bern, Bern, Switzerland.
出版信息
EMBO J. 2016 Apr 15;35(8):798-800. doi: 10.15252/embj.201694117. Epub 2016 Mar 11.
Abstract
The precise contribution of endoplasmic reticulum (ER) chaperone protein disulfide isomerase (PDI) variants in human amyotrophic lateral sclerosis (ALS) patients to the pathogenesis of ALS remained unclear. In the present study, Woehlbier (2016) demonstrated that these PDI variants are capable of altering motor neuron morphology, impairing the expression of synaptic proteins, and compromising neuromuscular junction (NMJ) integrity.
摘要
内质网（ER）伴侣蛋白二硫键异构酶（PDI）变体对人类肌萎缩侧索硬化症（ALS）患者发病机制的确切作用仍不清楚。在本研究中，Woehlbier（2016年）证明这些PDI变体能够改变运动神经元形态，损害突触蛋白的表达，并破坏神经肌肉接头（NMJ）的完整性。
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本文引用的文献
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