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Neuropathology of Bouncer Long Evans, a Novel Dysmyelinated Rat

机译:新型的脱髓鞘大鼠Bouncer Long Evans的神经病理学

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Backgroundandpurpose:Spontaneousanimalmutantsaffectedbyabnormalformationofmyelininthecentralnervoussystem(CNS)areusefulinstudiesonmyelinogenesisandremyelinationleadingtobetterunderstandingofcellularandmolecularinteractionsinvolvedinmyelinrepair.Anovelratmutant,BouncerLongEvans(LE-bo)isseverelydysmyelinated,butwithexceptionallongevity,anditsclinicalandpathologicphenotypearedescribed.brxmlns="http://pub2web.metastore.ingenta.coms/"/brMethods:Clinicalobservations,geneticstudies,anddeterminationoflongevitywereperformedinacolonyofrats,includingcarriersofLE-bophenotypeproducingthemutantanimals.Comprehensivehistologicstudieswereperformedonallperfusion-fixedtissues,andultrastructuralexaminationoftheopticnerveandthoracicpartofthespinalcordalsowasdoneinrats1to14weeksold.brxmlns="http://pub2web.metastore.ingenta.coms/"/brResults:TheLE-bophenotypeischaracterizedbywholebodytremor,progressivelysevereataxia,andsevereseizureactivity.TheLE-bophenotypeistransferredasanautosomalrecessivetraitandisstable.TheLE-boratcansurviveingoodhealthbeyond45weeks.Neuropathologicchangesincludesevereglobaldysmyelination,withthinuncompactedmyelinsheathsinyoungratsformingnomajordenseline,whereasthemyelinsheathsoftheperipheralnervoussystemappearnormal.Oligodendrocytesdegeneratewithapparentlyprogressingaccumulationofmembranousmaterialintheperikaryon.LargenumbersofimmatureglialcellsweredetectedintheCNSofLE-boratsat4to14weeks.brxmlns="http://pub2web.metastore.ingenta.coms/"/brConclusion:TheLE-boratisseverelydysmyelinatedduetoinabilityofitsoligodendrocytestoformmyelinsheaths.SimilaritiesoftheLE-boratandLongEvansShaker(les)ratneuropathologicfeatures,suchasseveredysmyelination,lackofmajordenselineinuncompactedmyelinsheaths,apparentproliferationofoligodendroglialcells,andconsiderablelongevity,arestrikingandsuggestthataLE-bomutationmayfunctionallyaffectthemyelinbasicproteingene.
机译:半边是我ndpurpose:Spontaneousanimalmutantsaffectedbyabnormalformationofmyelininthecentralnervoussystem(CNS)areusefulinstudiesonmyelinogenesisandremyelinationleadingtobetterunderstandingofcellularandmolecularinteractionsinvolvedinmyelinrepair.Anovelratmutant,BouncerLongEvans(LE-BO)isseverelydysmyelinated,butwithexceptionallongevity,anditsclinicalandpathologicphenotypearedescribed 方法:Clinicalobservations, geneticstudies,anddeterminationoflongevitywereperformedinacolonyofrats,簇集成振动贝里ersofLE-bophenotypeproducingthemutantanimals.Comprehensivehistologicstudieswereperformedonallperfusion-fixedtissues,andultrastructuralexaminationoftheopticnerveandthoracicpartofthespinalcordalsowasdoneinrats1to14weeksold 结果:R-bophenotypeischaracterizedbywholebodytremor,亲GRE委员会产生NYSE产生一个重新共济失调,andsevereseizureactivity.TheLE-表现型转移为常染色体隐性遗传我rasstavanie ble.TheLE-boratcansurviveingoodhealthbeyond45weeks.Neuropathologicchangesincludesevereglobaldysmyelination,命令和NU推荐路口ICT浓度为贪Bhangrapunjabi SFO蛭石马格诺可能条目寻找阿伯丁nseline,轴距记录被拒绝很可能Fudgy在PE里PHE RA巴尔内阿里奥坎伺服ssyste流体阿诺缅甸l.O暴力,直到Android的莱特DrossRotzank当局RA TE无线采取阿帕杜莱重新ntlypro温室委员会不累计有一个FME胸罩和SMA征用已平均PE里n.La乔治NU可靠个人接入被告人涂重新阿布鲁不Dolceacqua的llswe重新德特ICT集成ntheCNSofLE-boratsat4to14weeks 预告:R-boratisseverelydysmyelinatedduetoinabilityofitsoligodendrocytestoformmyelinsheaths.SimilaritiesoftheLE- boratandLongEvansShaker(les)大鼠的神经病理学特征,如髓鞘变性,未压实的髓鞘缺乏主要的密集线,少突胶质细胞的明显增殖以及相当长的寿命,在其中存在着明显的突变。

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