Behnke V, Langmann T
Lehrstuhl für Experimentelle Immunologie des Auges, Zentrum für Augenheilkunde, Medizinische Fakultät und Uniklinik Köln, Joseph-Stelzmann-Str. 9, 50931, Köln, Deutschland.
Zentrum für Molekulare Medizin, Köln, Deutschland.
Ophthalmologe. 2021 Feb;118(2):98-105. doi: 10.1007/s00347-020-01301-4. Epub 2021 Jan 7.
Retinal degeneration and neuroinflammation are often early hallmarks of different subtypes of neuronal ceroid lipofuscinosis (NCL) in patients and genetic animal models.
This article gives a summary of recently published research articles and novel concepts in the field of NCL-related neuroinflammation.
A search was carried out in PubMed for relevant publications and the results as well as own NCL-related research are discussed.
Microglia and other glial cells are chronically activated and show various dysfunctions in the central nervous system (CNS) and retina of NCL patients and animal models. This is accompanied by significant changes in the transcriptome and proteome. In NCL there is also involvement of the adaptive immune response, as demonstrated by the influx of autoantibodies and activated T cells.
A deeper understanding of the molecular processes that contribute to neuroinflammation and ultimately lead to neuronal cell death is an important basis for the discovery of possible biomarkers and the development of immunotherapies in NCL.
视网膜变性和神经炎症通常是患者及基因动物模型中不同亚型神经元蜡样脂褐质沉积症（NCL）的早期特征。
本文总结了NCL相关神经炎症领域最近发表的研究文章和新概念。
在PubMed中搜索相关出版物，并讨论结果以及自身与NCL相关的研究。
在NCL患者和动物模型的中枢神经系统（CNS）和视网膜中，小胶质细胞和其他胶质细胞长期被激活并表现出各种功能障碍。这伴随着转录组和蛋白质组的显著变化。在NCL中，适应性免疫反应也有参与，自身抗体和活化T细胞的流入证明了这一点。
深入了解导致神经炎症并最终导致神经元细胞死亡的分子过程，是发现NCL可能的生物标志物和开发免疫疗法的重要基础。
