谷氨酰胺转移酶6与神经系统疾病的研究进展  

The Research Progress of Relationship between Transglutaminase 6 and Neurologic Diseases

作　　者：陈葵 孙一忞 王坚 CHEN Kui;SUN Yi-min;WANG Jian(Department of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of Neurology,Shanghai Ninth People's Hospital,Shanghai Jiaotong University School of Medicine,Shanghai 200011,China)

机构地区：[1]复旦大学附属华山医院神经内科,上海200040 [2]上海交通大学医学院附属第九人民医院神经内科,上海200011

出　　处：《中国临床神经科学》2018年第6期707-711,共5页Chinese Journal of Clinical Neurosciences

摘　　要：谷氨酰胺转移酶6在脑内分布较多,能够催化蛋白质内部及分子间的交联,被认为与多种神经系统疾病相关,如谷蛋白共济失调、多发性硬化、脊髓小脑性共济失调等。其致病机制可能包括催化蛋白底物异常交联和自身免疫反应产生谷氨酰胺转移酶抗体等。近年来,编码谷氨酰胺转移酶6的TGM6基因被确认为脊髓小脑性共济失调35(SCA35)的致病基因,受到业内学者们越来越多的关注。文中重点介绍了谷氨酰胺转移酶6在神经系统疾病方面的研究现状,以及可能的致病机制。Transglutaminase 6is abundantly distributed in the brain and catalyze the intra-and intercrosslink of proteins,considered to be correlated with several neurological diseases,such as gluten ataxia, multiple sclerosisand spinocerebellar ataxia.Several mechanisms are supposed to be involved,for example, the autoimmune reaction producing anti-transglutaminase antibodies and the abnormal crosslinking of the substrates.In recent years,the TGM6 gene encoding transglutaminase 6 has been identified as a causative gene of spinocerebellar ataxia 35(SCA35),attracting more and more attention.This review focused on the current research progress between transglutaminase 6and neurological diseases,and the possible mechanisms.

关 键 词：谷氨酰胺转移酶6 神经系统疾病 神经退行性疾病 脊髓小脑性共济失调 

分 类 号：R742[医药卫生—神经病学与精神病学] Q189[医药卫生—临床医学]