title: Downregulation of genes involved in metabolism and oxidative stress in the peripheral leukocytes of Huntington's disease patients.
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 animal models. Here we examined if the expression changes could be visualized in the peripheral leukocytes of HD patients and pre-symptomatic HD (PreHD) carriers.
  
  
  RESULTS
  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, OXCT1 and CAP1, demonstrated consistent downregulation in peripheral leukocytes of both PreHD carriers and HD patients, while UCP2 was only down-regulated in HD patients.
  
  
  CONCLUSIONS
  These results provide potential peripheral biomarkers to indicate disease onset in preclinical stage, and to monitor the efficacy of early treatment. Further studies of a large series of preHD carriers and symptomatic HD patients will be warranted to verify the findings and examine if these markers correlate with clinical features.
authors.affiliation: Department of Neurology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taipei, Taiwan.
authors.forename: Kuo-Hsuan
authors.initials: KH
authors.lastname: Chang
authors.forename: Yi-Chun
authors.initials: YC
authors.lastname: Chen
authors.forename: Yih-Ru
authors.initials: YR
authors.lastname: Wu
authors.forename: Wan-Fen
authors.initials: WF
authors.lastname: Lee
authors.forename: Chiung-Mei
authors.initials: CM
authors.lastname: Chen
mesh_terms: D000154:Aconitate Hydratase / Q000235:genetics / Q000378:metabolism; D043383:Adenosylhomocysteinase / Q000235:genetics / Q000378:metabolism; D000328:Adult; D000818:Animals; D015415:Biomarkers / Q000378:metabolism; D016022:Case-Control Studies; D018797:Cell Cycle Proteins / Q000235:genetics / Q000378:metabolism; D015256:Coenzyme A-Transferases / Q000235:genetics / Q000378:metabolism; D003598:Cytoskeletal Proteins / Q000235:genetics / Q000378:metabolism; D015536:Down-Regulation; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D007473:Ion Channels / Q000235:genetics / Q000378:metabolism; D007963:Leukocytes, Mononuclear / Q000378:metabolism*; D008297:Male; D051379:Mice; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism; D018384:Oxidative Stress; D059467:Transcriptome; D000071246:Uncoupling Protein 2; D055815:Young Adult
chemical_list: D015415:Biomarkers; C489549:CAP1 protein, human; D018797:Cell Cycle Proteins; D003598:Cytoskeletal Proteins; D007473:Ion Channels; D024101:Mitochondrial Proteins; C000604389:UCP2 protein, human; C000604390:Ucp2 protein, mouse; D000071246:Uncoupling Protein 2; D015256:Coenzyme A-Transferases; C021460:3-ketoacid CoA-transferase; D043383:Adenosylhomocysteinase; C476079:ACO2 protein, human; D000154:Aconitate Hydratase