摘要
Transmissiblespongiformencephalopathyorpriondiseaseistriggeredbytheconversionfromcellularprionproteintopathogenicprionprotein.Growingevidencehasconcentratedonprionproteinconfigurationchangesandtheircorrelationwithpriondiseasetransmissibilityandpathogenicity.Invivoandinvitrostudieshaveshownthatseveralcytosolicformsofprionproteinwithspecifictopologicalstructurecandestroyintracellularstabilityandcontributetoprionproteinpathogenicity.Inthisstudy,thelatestmolecularchaperonesystemassociatedwithendoplasmicreticulum-associatedproteindegradation,theendoplasmicreticulumresidentproteinquality-controlsystemandtheubiquitinationproteasomesystem,isoutlined.Themolecularchaperonesystemdirectlycorrelateswiththeprionproteindegradationpathway.Understandingthemolecularmechanismswillhelpprovideafascinatingavenueforfurtherinvestigationsonpriondiseasetreatmentandprionprotein-inducedneurodegenerativediseases.
出版日期
2013年12月22日(中国期刊网平台首次上网日期,不代表论文的发表时间)