中华医学遗传学杂志
中華醫學遺傳學雜誌
중화의학유전학잡지
Chinese Journal of Medical Genetics
2015年
4期
562-566
,共5页
刘振%曾俊晟%曾胜%唐北沙%王俊岭
劉振%曾俊晟%曾勝%唐北沙%王俊嶺
류진%증준성%증성%당북사%왕준령
谷氨酰胺转氨酶%神经退行性疾病
穀氨酰胺轉氨酶%神經退行性疾病
곡안선알전안매%신경퇴행성질병
Transglutaminase%Neurodegenerative disease
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.
穀氨酰胺轉胺酶(transglutaminase,TGase或TG)是一類Ca2+依賴酶.在齧齒動物和人體內已髮現的TGase傢族包括TG1~7、血漿凝血因子ⅩⅢa和紅細胞膜蛋白4.2等共9種,其中前8種具有催化活性.TG可催化穀氨酰氨基的各種轉化反應,包括轉酰胺基作用、脫酰胺基作用和酯化作用,併參與蛋白質多肽穀氨酰胺殘基和賴氨酰殘基之間的交聯反應等蛋白轉錄後脩飾,穩定蛋白結構,催化蛋白聚集體的形成.目前已髮現TGase參與多種重要的生理病理過程,影響多種疾病的髮生和髮展,尤其是亨廷頓病、脊髓小腦型共濟失調、阿爾茨海默病、帕金森病等神經繫統退行性疾病與TGase在人體內的作用密切相關.
곡안선알전알매(transglutaminase,TGase혹TG)시일류Ca2+의뢰매.재교치동물화인체내이발현적TGase가족포괄TG1~7、혈장응혈인자ⅩⅢa화홍세포막단백4.2등공9충,기중전8충구유최화활성.TG가최화곡안선안기적각충전화반응,포괄전선알기작용、탈선알기작용화지화작용,병삼여단백질다태곡안선알잔기화뢰안선잔기지간적교련반응등단백전록후수식,은정단백결구,최화단백취집체적형성.목전이발현TGase삼여다충중요적생리병리과정,영향다충질병적발생화발전,우기시형정돈병、척수소뇌형공제실조、아이자해묵병、파금삼병등신경계통퇴행성질병여TGase재인체내적작용밀절상관.
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 Ⅲ 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 lysylresidue,stabilizing protein structure and catalyzing formation of protein aggregates.TGase has been found to contribute to a variety of 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.