中国神经再生研究(英文版) ›› 2015, Vol. 10 ›› Issue (10): 1563-1564.doi: 10.4103/1673-5374.165263

• 观点:神经损伤修复保护与再生 • 上一篇    下一篇

控制细胞返变与再生:基因表达“开关”? 

  

  • 收稿日期:2015-06-29 出版日期:2015-10-28 发布日期:2015-10-28
  • 基金资助:

    这项工作由德意志研究联合会(DFG授予EL125/6-1)支持

Schwann cell Miz without POZ: degeneration
meets regeneration

David Fuhrmann, Hans-Peter Elsässer*   

  1. Department of Cytobiology and Cytopathobiology, Philipps University of Marburg, Robert-Koch-Strasse 6, 35033 Marburg, Germany
  • Received:2015-06-29 Online:2015-10-28 Published:2015-10-28
  • Contact: Hans-Peter Els?sser, Ph.D.,elsaesse@mailer.uni-marburg.de.
  • Supported by:

    This work was supported by Deutsche Forschungsgemeinschaft (DFG grant EL125/6-1).

摘要:

周围运动和感觉神经病变是具有不同病因的疾病,病因包括遗传性疾病,糖尿病,感染或炎症,或酗酒中毒引起。遗传性神经病变,以进行神经性腓骨肌萎缩病最为常见。根据结构和电生理特性,肌骨肌萎缩分为轴突和脱髓鞘形式。周髓鞘蛋白22、髓鞘蛋白零和连接蛋白32在人类脱髓鞘性神经病中是最常受到影响的基因,在细胞中转录因子Myc相互作用锌丹蛋白Miz防缺失的小鼠的模型中。目前已经设计出过重动物模型来模拟这些人类疾病,这些模型依赖于与人类神经病变相关基因的缺失,过表达或突变,通常的临床症状会在早期发生,并且病程呈现渐进性特点。近日,桑斯莫雷诺等人公布的转录因子Myc相互作用的小鼠许旺细胞锌指蛋白1(Miz1)部分缺失,我们观察到迟发脱髓鞘神经病变伴随自发临床缓解,从而为研究周围神经再生引进一种独特的新模型,我们对其进行了详细分析和研究。

Abstract:

Peripheral motor and sensory neuropathies are diseases with different etiologies emerging from genetic disorders, diabetes, infection or inflammation, paraneoplastic damage or intoxications including alcohol abuse. Hereditary neuropathies, most of which are subsumed under the hypernym Charcot-Marie Tooth (CMT) disease, are clinically heterogeneous: about 50 affected gene loci encoding about 30 genes have been so far identified to be involved in the pathogenesis of different CMTs. Depending on structural and electrophysiological features CMTs are divided into axonal and demyelinating forms. Peripheral myelin protein 22 (PMP22), myelin protein zero (MPZ) and connexin 32 (GJB1) are the most frequently affected genes in human demyelinating neuropathies and a variety of animal models have been designed to mimic these human disorders. These models rely on the deletion, overexpression or mutation of the gene relevant for a human neuropathy and usually clinical symptoms occur early in life and the course of disease is progressive. Recently, Sanz-Moreno et al. published the partial deletion of the transcription factor Myc interacting zinc finger protein 1 (Miz1) in Schwann cells of mice and observed a late onset demyelinating neuropathy with a spontaneous clinical remission, introducing a unique new model to study peripheral nerve regeneration.