Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntingtons Disease 英文参考文献.docVIP

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Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntingtons Disease 英文参考文献.doc

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Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntingtons Disease 英文参考文献

FormationofPolyglutamineInclusionsinaWideRange ofNon-CNSTissuesintheHdhQ150Knock-InMouse ModelofHuntington’sDisease HilaryMoffitt1,GrahamD.McPhail2,BenWoodman1,CarlHobbs3,GillianP.Bates1* 1DepartmentofMedicalandMolecularGenetics,King’sCollegeLondonSchoolofMedicine,London,UnitedKingdom,2DivisionofCellularPathology,Bartsandthe London NHS Trust, Royal London Hospital, London, United Kingdom, 3Wolfson Centre for Age-Related Diseases, Kings CollegeLondon School of Medicine, London, UnitedKingdom Abstract Background: Huntington’s disease (HD) is an inherited progressive neurodegenerative disorder caused by a CAG repeat expansionintheubiquitouslyexpressedHDgeneresultinginanabnormallylongpolyglutaminerepeatinthehuntingtin protein.Polyglutamineinclusions areahallmarkoftheneuropathologyofHD.Wehavepreviouslyshownthatinclusion pathology is also present in the peripheral tissues of the R6/2 mouse model of HD which expresses a small N-terminal fragment of mutant huntingtin. To determine whether this peripheral pathology is a consequence of the aberrant expression of this N-terminal fragment, we extend this analysis to the genetically precise knock-in mouse model of HD, HdhQ150,whichexpressesmutantmousehuntingtin. Methodology/PrincipalFindings:WehavepreviouslystandardizedtheCAGrepeatsizeandstrainbackgroundoftheR6/2 and HdhQ150 knock-in mouse models and found that they develop a comparable and widespread neuropathology. To determinewhetherHdhQ150knock-inmicealsodevelopperipheralinclusionpathology,homozygousHdhQ150/Q150 mice wereperfusionfixedat22monthsofage,andtissueswereprocessedforhistologyandimmunohistochemistrywiththe anti-huntingtinantibodyS830.TheperipheralinclusionpathologywasalmostidenticaltothatfoundinR6/2miceat12 weeksofagewithminordifferencesininclusionabundance. Conclusions/Significance: The highly comparable peripheral inclusion pathology that is present in both the R6/2 and HdhQ150knock-inmodelsofHDindicatesthatthepresenceofperipheralinclusionsinR6/2miceisnotaconse