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研究囊性纤维化的新途径

2009-12-01 www.91sqs.com A +

美国科研人员培育出来了能和人类同样罹患囊肿性纤维化疾病的小猪,旨在更快找到治愈该疾病的良方。

爱荷华州的研究人员表示,他们培育出了基因发生变异而导致患囊肿性纤维化疾病的小猪。

爱荷华州的沃尔什(MichaelWelsh)博士表示,研究囊肿性纤维化病的发展进程可以让人们更多了解其信息,并有助于探寻新的治疗方法,目前科学家还对囊肿性纤维化的成因一无所知。

为研究募集资金的囊肿性纤维化基金表示,全世界有7万人患囊肿性纤维化疾病,其中3万人在美国。患者最多能活到37岁左右。

研究人员选择猪的理由是猪肺与人肺有很多相似之处。

用小猪做实验可以模拟囊肿性纤维化在人身上发病时的最初症状,研究人员希望等到疾病在猪肺彻底形成,“这样我们就可以开展以前不可能完成的一些实验了”。

U.S.scientistshavecreatedpigsthatappeartodevelopcysticfibrosisjustaspeopledo,asteptheyhopewillaccelerateeffortstotacklethedisease.

WritinginthejournalScienceonThursday,scientistsattheUniversityofIowaandtheUniversityofMissourisaidtheycreatedgeneticallyengineeredpigletswiththesamemutationthatcausescysticfibrosisinpeople.

Studyinghowthediseaseunfoldsinthesepigsmayprovideinsightsintocysticfibrosisthatthusfarhaveeludedscientistsandcouldpointtowardnewtreatmentsormaybeevenacure,saidDr.MichaelWelshoftheUniversityofIowa.

PeoplegetCFwhentheyinherittwomutatedcopiesofagenecalledCFTR,whichwaspinpointedasthecausein1989.Thediseasecausesmucustoaccumulateandclogsomeoftheorgansinthebody,especiallythelungsandpancreas.

TheCysticFibrosisFoundation,whichhelpedfundthestudy,saidabout70,000peopleworldwide,including30,000peopleintheUnitedStates,haveCF.Peoplewiththediseasecanexpecttolivetoaboutage37.

Scientistscreateanimal"models"ofadiseaseinordertoperformexperimentsthatwouldnotbepossiblewithpeople.Micewiththegeneticabnormalitythatcausescysticfibrosishavebeendeveloped,butthediseasepresentsitselfverydifferentlyintheserodentsthaninpeople.

"Unfortunately,themiceleavesomethingtobedesired,"Welsh,whohelpedleadthestudy,saidinatelephoneinterview.

"Theydon'tgetthepancreaticdiseaselikepeoplewithCFget.Theydon'tgetthelungdiseaselikepeoplewithCFget.Theydon'tgettheintestinaldiseaselikepeoplewithCFget.There'sbeenmanyquestionsthatcan'tbeanswered,"headded.

"Theonsetoftheseproblemsisdifficulttotrackdowninhumansbecausesometimestheyhappenbeforebirthorsometimestheyhappensilently,"addedChristopherPenlandofthefoundation.

Thisisthelatestexampleofpigsusedforhumanmedicalneeds.Forexample,cardiacpatientscangetpigheartvalves.

Thescientistsdevelopedpigswithcysticfibrosisbecausetheirlungshavemanycharacteristicsofhumanlungs.

"Rightnow,ifyouwanttodoexperimentstofindtreatmentsortherapiesforthelungdiseasethatisfatalforpeoplewithCF,youwouldhavetoexperimentonkidsthathaveCF,"RandyPratheroftheUniversityofMissouriadded.

Thediseaseinpigscloselymimickedtheinitialstagesofthediseaseseeninpeople.ThescientistsarewaitingforthemtodeveloplungdiseasetypicalofCF"sowecanstartexperimentinginwaysthathaveneverbeenpossible,"Prathersaidinastatement.



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