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Cell Cycle Deregulation in Ewings Sarcoma Pathogenesis英文文献资料
HindawiPublishingCorporation
Sarcoma
Volume2011,ArticleID598704,10pages
doi:10.1155/2011/598704
ReviewArticle
CellCycleDeregulationinEwing’sSarcomaPathogenesis
AshleyA.Kowalewski,
1R.LorRandall,2,3andStephenL.Lessnick1,2,4
1
2
3
4
DepartmentofOncologicalSciences,UniversityofUtahSchoolofMedicine,2000CircleofHope,
Room4242,SaltLakeCity,UT84112,USA
CenterforChildren’sCancerResearch,HuntsmanCancerInstitute,UniversityofUtahSchoolofMedicine,
2000CircleofHope,Room4242,SaltLakeCity,UT84112,USA
SarcomaServices,DepartmentofOrthopaedics,UniversityofUtahSchoolofMedicine,2000CircleofHope,
Room4242,SaltLakeCity,UT84112,USA
DivisionofPediatricHematology/Oncology,UniversityofUtahSchoolofMedicine,2000CircleofHope,
Room4242,SaltLakeCity,UT84112,USA
CorrespondenceshouldbeaddressedtoStephenL.Lessnick,stephen.lessnick@
Received29June2010;Accepted7October2010
AcademicEditor:R.Pollock
Copyright?2011AshleyA.Kowalewskietal.ThisisanopenaccessarticledistributedundertheCreativeCommonsAttribution
License,whichpermitsunrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalworkisproperly
cited.
Ewing’ssarcomaisahighlyaggressivepediatrictumorofbonethatusuallycontainsthecharacteristicchromosomaltranslocation
t(11;22)(q24;q12).ThistranslocationencodestheoncogenicfusionproteinEWS/FLI,whichactsasanaberranttranscription
factortoderegulatetargetgenesnecessaryforoncogenesis.Onekeyfeatureofoncogenictransformationisdysregulationofcell
cyclecontrol.ItisthereforelikelythatEWS/FLIandothercooperatingmutationsinEwing’ssarcomamodulatethecellcycleto
facilitatetumorigenesis.ThispaperwillsummarizecurrentpublisheddataassociatedwithderegulationofthecellcycleinEwing’s
sarcomaandhighlightimportantquestionsthatremaintobeanswered.
1.Introduction
the 5
portion of FLI1 from chromosome 11 [8, 9]. EWSR1
portion of EWSR1 from chromosome 22 with the
3
FirstdescribedbyJamesEwingin1921,Ewing’ssarcomais
characterizedasahighlyaggressive,undi?erentiatedtumor
of the bone [1]. Although it is the second most
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