Biological Role of Dystroglycan in Schwann Cell Function and Its Implications in Peripheral Nervous System Diseases英文文献资料.docVIP

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Biological Role of Dystroglycan in Schwann Cell Function and Its Implications in Peripheral Nervous System Diseases英文文献资料.doc

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Biological Role of Dystroglycan in Schwann Cell Function and Its Implications in Peripheral Nervous System Diseases英文文献资料

HindawiPublishingCorporation JournalofBiomedicineandBiotechnology Volume2010,ArticleID740403,17pages doi:10.1155/2010/740403 ReviewArticle BiologicalRoleofDystroglycaninSchwannCellFunctionand ItsImplicationsinPeripheralNervousSystemDiseases ToshihiroMasaki1,2andKiichiroMatsumura3 1 CentreforNeuroregeneration,UniversityofEdinburgh,Chancellor’sBuilding,49LittleFranceCrescent, EdinburghEH164SB,UK 2 3 FacultyofMedicalSciences,TeikyoUniversityofScience,2-2Senju-Sakuragi,Adachi-ku,Tokyo120-0045,Japan DepartmentofNeurologyandNeuroscience,TeikyoUniversity,2-11-1Kaga,Itabashi-ku,Tokyo173-8605,Japan CorrespondenceshouldbeaddressedtoToshihiroMasaki,t-masaki@ntu.ac.jp Received22October2009;Accepted20April2010 AcademicEditor:Chung-LiangChien Copyright?2010T.MasakiandK.Matsumura. This is an open access article distributed under the Creative Commons AttributionLicense,whichpermitsunrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalworkis properlycited. Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC) that links extracellular matrix with cytoskeleton,expressedinavarietyoffetalandadulttissues.Dystroglycanplaysdiverserolesindevelopmentandhomeostasis includingbasementmembraneformation,epithelialmorphogenesis,membranestability,cellpolarization,andcellmigration. Inthispaper,wewillfocusonbiologicalroleofdystroglycaninSchwanncellfunction,especiallymyelination.First,wereview themoleculararchitectureofDGCinSchwanncellabaxonalmembrane.Then,wewillreviewtheloss-of-functionstudiesusing targetedmutagenesis,whichhaverevealedbiologicalfunctionsofeachcomponentofDGCinSchwanncells.Basedonthese ?ndings, roles of dystroglycan in Schwann cell function, in myelination in particular, and its implications in diseases will be discussedindetail.Finally,inviewofthefactthatunderstandingtheroleofdystroglycaninSchwanncellsisjustbeginning,future perspectiveswillbediscussed. 1.Introduction been implicated in basal lamina development in mouse embryo [5], e