Downregulation of Genes Involved in Metabolism and Oxidative Stress in the Peripheral Leukocytes of Huntingtons Disease Patients 英文参考文献.docVIP

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Downregulation of Genes Involved in Metabolism and Oxidative Stress in the Peripheral Leukocytes of Huntingtons Disease Patients 英文参考文献.doc

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Downregulation of Genes Involved in Metabolism and Oxidative Stress in the Peripheral Leukocytes of Huntingtons Disease Patients 英文参考文献

DownregulationofGenesInvolvedinMetabolismand OxidativeStressinthePeripheralLeukocytesof Huntington’sDiseasePatients Kuo-HsuanChang,Yi-ChunChen,Yih-RuWu,Wan-FenLee,Chiung-MeiChen* DepartmentofNeurology,ChangGungMemorialHospital,ChangGungUniversityCollegeofMedicine,Taipei,Taiwan Abstract Background: Huntington’s disease (HD) is caused by expanded CAG repeats encoding a polyglutamine tract in the huntingtin (HTT) protein. A number of differentially-expressed protein molecules have been identified in striatum of HD animalmodels.HereweexaminediftheexpressionchangescouldbevisualizedintheperipheralleukocytesofHDpatients andpre-symptomaticHD(PreHD)carriers. Methods and findings: The expression levels of 17 candidate genes that differentially expressed in striatum between transgenic HD and wild-type mice in literature were measured in the peripheral leukocytes of 4 PreHD carriers, 16 HD patients and 20 healthy controls. Four genes majorly involved in metabolism and oxidative stress response, including AHCY1,ACO2,OXCT1andCAP1,demonstratedconsistentdownregulationinperipheralleukocytesofbothPreHDcarriers andHDpatients,whileUCP2wasonlydown-regulatedinHDpatients. Conclusion: These results provide potential peripheral biomarkers to indicate disease onset in preclinical stage, and to monitortheefficacyofearlytreatment.FurtherstudiesofalargeseriesofpreHDcarriersandsymptomaticHDpatientswill bewarrantedtoverifythefindingsandexamineifthesemarkerscorrelatewithclinicalfeatures. Citation: Chang K-H, Chen Y-C, Wu Y-R, Lee W-F, Chen C-M (2012) Downregulation of Genes Involved in Metabolism and Oxidative Stress in the Peripheral LeukocytesofHuntington’sDiseasePatients.PLoSONE7(9):e46492.doi:10.1371/journal.pone.0046492 Editor:DavidR.Borchelt,UniversityofFlorida,UnitedStatesofAmerica ReceivedJune11,2012;AcceptedSeptember5,2012;PublishedSeptember27,2012 Copyright: ? 2012 Chang et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which