title: Increased oxidative damage and mitochondrial abnormalities in the peripheral blood of Huntington's disease patients.
abstract: Increased oxidative stress and mitochondrial abnormalities contribute to neuronal dysfunction in Huntington's disease (HD). We investigated whether these pathological changes in HD brains may also be present in peripheral tissues. Leukocyte 8-hydroxydeoxyguanosine (8-OHdG) and plasma malondialdehyde (MDA) were elevated, and activities of erythrocyte Cu/Zn-superoxide dismutase (Cu/Zn-SOD) and glutathione peroxidase (GPx) reduced in 16 HD patients when compared to 36 age- and gender-matched controls. Deleted and total mitochondrial DNA (mtDNA) copy numbers were increased, whereas the mRNA expression levels of mtDNA-encoded mitochondrial enzymes are not elevated in HD leukocytes compared to the normal controls. Plasma MDA levels also significantly correlated with HD disease severity. These results indicate means to suppress oxidative damage or to restore mitochondrial functions may be beneficial to HD patients. Plasma MDA may be used as a potential biomarker to test treatment efficacy in the future, if confirmed in a larger, longitudinal study.
authors.affiliation: Department of Neurology, Chang Gung Memorial Hospital, Chang-Gung University College of Medicine, Taipei, Taiwan. cmchen@adm.cgmh.org.tw
authors.forename: Chiung-Mei
authors.initials: CM
authors.lastname: Chen
authors.forename: Yih-Ru
authors.initials: YR
authors.lastname: Wu
authors.forename: Mei-Ling
authors.initials: ML
authors.lastname: Cheng
authors.forename: Jun-Liang
authors.initials: JL
authors.lastname: Liu
authors.forename: Yu-May
authors.initials: YM
authors.lastname: Lee
authors.forename: Po-Wei
authors.initials: PW
authors.lastname: Lee
authors.forename: Bing-Wen
authors.initials: BW
authors.lastname: Soong
authors.forename: Daniel Tsun-Yee
authors.initials: DT
authors.lastname: Chiu
mesh_terms: D000080242:8-Hydroxy-2'-Deoxyguanosine; D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000378:metabolism; D004272:DNA, Mitochondrial / Q000378:metabolism; D003849:Deoxyguanosine / Q000031:analogs & derivatives / Q000494:pharmacology; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000097:blood*; D008297:Male; D008315:Malondialdehyde / Q000097:blood; D008875:Middle Aged; D008928:Mitochondria / Q000378:metabolism*; D018384:Oxidative Stress; D013482:Superoxide Dismutase / Q000097:blood
chemical_list: D015415:Biomarkers; D004272:DNA, Mitochondrial; D008315:Malondialdehyde; D000080242:8-Hydroxy-2'-Deoxyguanosine; D013482:Superoxide Dismutase; D003849:Deoxyguanosine