Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis  

作　　者：Francisco J.Barrantes 

机构地区：[1]Biomedical Research Institute(BIOMED)UCA-CONICET,Buenos Aires,Argentina

出　　处：《Neural Regeneration Research》2021年第2期242-246,共5页中国神经再生研究（英文版）

摘　　要：Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles.The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction,the nicotinic acetylcholine receptor.Other variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral synapse.This review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia gravis.The review also covers the application of a powerful biophysical technique,superresolution optical microscopy,to image the nicotinic receptor in live cells and follow its motional dynamics.The hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor,as observed in myasthenia gravis.

关 键 词：AGRIN autoimmune diseases muscle end-plate muscle specific kinase MUSK myasthenia gravis NANOSCOPY neuromuscular junction nicotinic acetylcholine receptor RAPSYN superresolution microscopy 

分 类 号：R746.1[医药卫生—神经病学与精神病学]