典型文献
Elucidating the pathological mechanisms of neurodegeneration in the lethal serpinopathy FENIB
文献摘要：
The term serpinopathies was introduced to describe a family of diseases caused by point mutations in serine protease inhibitors, or serpins. Serpins inhibit their cognate protease by an irreversible suicide mechanism starting with the attack of the active site serine on the reactive center loop of the inhibitor, followed by formation of a covalent complex between both proteins, insertion of the reactive center loop of the serpin into its own beta-sheet A, and culminating in distortion of the active site of the serine protease and thus irreversible inactivation. This inhibitory mechanism, reminiscent of the movement of a mousetrap, requires a structural flexibility that proves to be unfavorable when the folding of the serpin is altered by mutations responsible for conformational rearrangements, allowing an intermolecular domain exchange characterized by the insertion of the C-terminal domain of a molecule into a second one, thus forming a dimer. Expansion of this insertion reaction leads to the formation of serpin polymers that accumulate within the endoplasmic reticulum (ER) of the cell and consequently reduces the secretion of the wild-type serpin (Greene et al., 2016).
文献关键词：
中图分类号：
作者姓名：
Elena Miranda;Giovanna Galliciotti
作者机构：
Department of Biology and Biotechnologies'Charles Darwin'and Pasteur Institute–Cenci Bo-lognetti Foundation,Sapienza University of Rome,Rome,Italy;Institute of Neuropathology,University Medical Center Hamburg-Eppendorf,Hamburg,Germany
文献出处：
引用格式：
[1]Elena Miranda;Giovanna Galliciotti-.Elucidating the pathological mechanisms of neurodegeneration in the lethal serpinopathy FENIB)[J].中国神经再生研究（英文版）,2022(08):1733-1734
A类：
serpinopathy,FENIB,serpinopathies,serpins,Serpins,mousetrap
B类：
Elucidating,pathological,mechanisms,neurodegeneration,lethal,was,introduced,describe,family,diseases,caused,by,point,mutations,serine,protease,inhibitors,their,cognate,irreversible,suicide,starting,attack,site,reactive,center,loop,followed,covalent,complex,between,both,proteins,insertion,into,its,own,beta,sheet,culminating,distortion,thus,inactivation,This,inhibitory,reminiscent,movement,requires,structural,flexibility,that,proves,unfavorable,when,folding,altered,responsible,conformational,rearrangements,allowing,intermolecular,domain,exchange,characterized,terminal,molecule,second,one,forming,dimer,Expansion,this,reaction,leads,polymers,accumulate,within,endoplasmic,reticulum,ER,cell,consequently,reduces,secretion,wild,type,Greene
AB值：
0.560903
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