谷氨酰胺转氨酶与神经退行性疾病  

Transglutaminase and neurodegenerative diseases

作　　者：刘振[1] 曾俊晟 曾胜[1] 唐北沙[1] 王俊岭[1] 

机构地区：[1]中南大学湘雅医院神经内科,长沙410008

出　　处：《中华医学遗传学杂志》2015年第4期562-566,共5页Chinese Journal of Medical Genetics

基　　金：“十二五”国家科技支撑计划项目（2012BA109B04）;卫生部公益性行业科研专项基金（201302001）;国家自然科学基金（81130021、81300981、81250015）;高等学校博士学科点专项科研基金（20120162120048）

摘　　要：谷氨酰胺转胺酶（transglutaminase，TGase或TG）是一类Ca2＋依赖酶。在啮齿动物和人体内已发现的TGase家族包括TG1～7、血浆凝血因子XⅢa和红细胞膜蛋白4．2等共9种，其中前8种具有催化活性。TG可催化谷氨酰氨基的各种转＋化反应，包括转酰胺基作用、脱酰胺基作用和酯化作用，并参与蛋白质多肽谷氨酰胺残基和赖氨酰残基之间的交联反应等蛋白转录后修饰，稳定蛋白结构，催化蛋白聚集体的形成。目前已发现TGase参与多种重要的生理病理过程，影响多种疾病的发生和发展，尤其是亨廷顿病、脊髓小脑型共济失调、阿尔茨海默病、帕金森病等神经系统退行性疾病与TGase在人体内的作用密切相关。Transglutaminase （TG） is a kind of calcium-dependent enzymes. The TGase family found in rodents and human contains 9 types, including TG1-7, blood coagulation factor X Ill a and erythrocyte membrane protein 4. 2, with the former 8 types possessing catalytic ：activity. TG catalyzes various conversion reactions of glutamine, including transamination, deamination and esterification, and participates in post-transcriptional modification of proteins such as cross-linking peptides glutamine residue and lysyl- residue, stabilizing protein structure and catalyzing formation of protein aggregates. TGase has been found to contribute to a variety o{ important physiological and pathological processes and play a role in the pathogenesis of multiple diseases. Notably, neurodegenerative diseases such as Huntington~ s disease, spinocerebellar ataxia, Alzheimer＇s disease and Parkinson＇s disease, have a close connection with TGase^s role in the human body.

关 键 词：谷氨酰胺转氨酶 神经退行性疾病 

分 类 号：R318.08[医药卫生—生物医学工程]