Drosophila Carrying Pex3 or Pex16 Mutations Are Models of Zellweger Syndrome That Reflect Its Symptoms Associated with the Absence of Peroxisomes 英文参考文献.docVIP

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Drosophila Carrying Pex3 or Pex16 Mutations Are Models of Zellweger Syndrome That Reflect Its Symptoms Associated with the Absence of Peroxisomes 英文参考文献.doc

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Drosophila Carrying Pex3 or Pex16 Mutations Are Models of Zellweger Syndrome That Reflect Its Symptoms Associated with the Absence of Peroxisomes 英文参考文献

DrosophilaCarryingPex3orPex16MutationsAreModels ofZellwegerSyndromeThatReflectItsSymptoms AssociatedwiththeAbsenceofPeroxisomes MinoruNakayama1,2,3,HiroyasuSato2,TakayukiOkuda2,NaoFujisawa2,NozomuKono4,HiroyukiArai4, EmikoSuzuki5,MasatoUmeda6,HiroyukiO.Ishikawa1,KenjiMatsuno1,2,3* 1GenomeandDrugResearchCenter,TokyoUniversityofScience,Noda,Chiba,Japan,2DepartmentofBiologicalScienceandTechnology,TokyoUniversityofScience, Noda, Chiba, Japan, 3Research Institute for Science and Technology, Tokyo University of Science, Noda, Chiba, Japan, 4Graduate School of Pharmaceutical Science, UniversityofTokyo,Hongo,Bunkyo-ku,Tokyo,Japan,5StructuralBiologyCenter,NationalInstituteofGenetics,andDepartmentofGenetics,TheGraduateUniversityfor AdvancedStudies,Mishima,Shizuoka,Japan,6InstituteforChemicalResearch,KyotoUniversity,Uji,Kyoto,Japan Abstract Theperoxisomebiogenesisdisorders(PBDs)arecurrentlydifficult-to-treatmultiple-organdysfunctiondisordersthatresult fromthedefectivebiogenesisofperoxisomes.GenesencodingPeroxins,whicharerequiredforperoxisomebiogenesisor functions, are known causative genes of PBDs. The human peroxin genes PEX3 or PEX16 are required for peroxisomal membraneproteintargeting,andtheirmutationscauseZellwegersyndrome,aclassofPBDs.Lackofunderstandingabout the pathogenesis of Zellweger syndrome has hindered the development of effective treatments. Here, we developed potentialDrosophilamodelsforZellwegersyndrome,inwhichtheDrosophilapex3orpex16genewasdisrupted.Asfoundin Zellwegersyndromepatients,peroxisomeswerenotobservedinthehomozygousDrosophilapex3mutant,whichwaslarval lethal.However,thepex16homozygotelackingitsmaternalcontributionwasviableandstillmaintainedasmallnumberof peroxisome-like granules, even though PEX16 is essential for the biosynthesis of peroxisomes in humans. These results suggestthattherequirementsforpex3andpex16inperoxisomebiosynthesisinDrosophilaaredifferent,andtheroleof PEX16orthologsmayhavedivergedbetweenmammalsandDrosophila.ThephenotypesofourZel