中国神经再生研究(英文版) ›› 2017, Vol. 12 ›› Issue (4): 562-565.doi: 10.4103/1673-5374.205086

• 综述:神经损伤修复保护与再生 • 上一篇    下一篇

运动神经元和CD4 + T细胞

  

  • 收稿日期:2017-02-28 出版日期:2017-04-15 发布日期:2017-04-15
  • 基金资助:

    此项工作由美国国立卫生研究院和国立神经病与中风研究所共同赞

A tale of motor neurons and CD4+ T cells: moving forward by looking back

Abhirami Kannan Iyer, Kathryn J. Jones   

  1. Department of Anatomy and Cell Biology, Indiana University School of Medicine, Indianapolis, IN, USA
  • Received:2017-02-28 Online:2017-04-15 Published:2017-04-15
  • Contact: Kathryn J. Jones, Ph.D., kjjones1@iupui.edu.
  • Supported by:

    This study was supported by grants from NIH/NINDS R01 funding NS40433.

摘要:

 

肌萎缩侧索硬化症(ALS)是以脑干、运动皮质和脊髓中的运动神经元丧失为特征的神经变性疾病。ALS 的临床特征为肌肉萎缩、进行性麻痹以及呼吸衰竭而亡。偶发性和家族性疾病共有临床和病理特征,表明常见的致病途径可能是运动神经元死亡。基因突变编码SOD1酶是首次被认定的家族性ALS的遗传原因。先天性和适应性免疫系统的激活在ALS神经变性疾病中被越来越多的研究证实。CD4+ T细胞被认为是神经变性疾病的病理驱动因素。

文章认为在了解ALS发病机制方面有很长的路要走,目前仅仅是理解了促进运动神经元存活所涉及的神经免疫相互作用。具体地,触发这种免疫应答的Ag、细胞源和MHC II类动力学以及辅助分子,以及Th2细胞支持运动神经元存活的机制,以及这种免疫介导的神经保护还尚未清楚。涉及CD4+ T细胞的研究将为发现ALS中易于获得的生物标志物提供信息,以便早期诊断、监测疾病和评估治疗效果。

ORCID:0000-0002-0580-5436(Kathryn J. Jones)

Abstract:

Amyotrophic lateral sclerosis (ALS) is a fatal progressive disorder characterized by the selective degeneration of motor neurons (MN). The impact of peripheral immune status on disease progression and MN survival is becoming increasingly recognized in the ALS research field. In this review, we briefly discuss findings from mouse models of peripheral nerve injury and immunodeficiency to understand how the immune system regulates MN survival. We extend these observations to similar studies in the widely used superoxide dismutase 1 (SOD1) mouse model of ALS. Last, we present future hypotheses to identify potential causative factors that lead to immune dysregulation in ALS. The lessons from preceding work in this area offer new exciting directions to bridge the gap in our current understanding of immune-mediated neuroprotection in ALS.

Key words: amyotrophic lateral sclerosis (ALS), superoxide dismutase 1 (SOD1), immune system, SOD1 mice, motor neuron, CD4+ T cells, neuroprotection