title: Association of status redox with demographic, clinical and imaging parameters in patients with Huntington's disease.
abstract: Huntington's disease (HD) is an autosomal dominant, progressive neurodegenerative disorder, caused by an expanded trinucleotide CAG sequence of the huntingtin (Htt) gene, which encodes a stretch of glutamines in the Htt protein. The mechanisms of neurodegeneration associated with the accumulation of Htt aggregates still remains unclear.
  
  
  OBJECTIVE
  To determine oxidative stress biomarkers in HD patients and their relationship with clinical, demographic and neuroimaging parameters.
  
  
  METHODS
  Fourteen patients and 39 controls paired by age and sex participated in this study. Oxidative damage was assayed in blood by measuring malondialdehyde (MDA) and advanced oxidative protein products (AOPPs). Antioxidant status was determined by activities of superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), glutathione reductase (GR), reduced glutathione (GSH), protein thiols and total antioxidant capacity (FRAP). The Unified Huntington Disease Rating Scale (UHDRS) and neuroimaging studies were also employed.
  
  
  RESULTS
  MDA, AOPP and GPx were significantly increased in HD patients with respect to the control group, while GR activity was decreased. FRAP correlated with age of disease onset, AOPP with motor severity (UHDRS score), age of patients and age of disease onset. Caudate atrophy was associated with lower plasma concentrations of GSH.
  
  
  CONCLUSIONS
  These findings point to a redox imbalance in HD patients. GR activity could be a potential biomarker for symptom onset in asymptomatic gene carriers, while plasmatic GSH could be useful in monitoring the progression of neurodegeneration - as an expression of caudate atrophy - during the course of the disease.
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: marisol.pena@infomed.sld.cu.
authors.forename: Marisol
authors.initials: M
authors.lastname: Peña-Sánchez
authors.affiliation: National Genetic Center, Cuba. Electronic address: gretel.riveron@infomed.sld.cu.
authors.forename: Gretel
authors.initials: G
authors.lastname: Riverón-Forment
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: tzv@infomed.sld.cu.
authors.forename: Tatiana
authors.initials: T
authors.lastname: Zaldívar-Vaillant
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: alexisl@infomed.sld.cu.
authors.forename: Alexis
authors.initials: A
authors.lastname: Soto-Lavastida
authors.affiliation: Faculty of Medicine "Calixto Garcia", Cuba. Electronic address: borrerojudith@yahoo.com.
authors.forename: Judith
authors.initials: J
authors.lastname: Borrero-Sánchez
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: glara@infomed.sld.cu.
authors.forename: Gloria
authors.initials: G
authors.lastname: Lara-Fernández
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: eesteban@infomed.sld.cu.
authors.forename: Enrique M
authors.initials: EM
authors.lastname: Esteban-Hernández
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: zmhernandez@infomed.sld.cu.
authors.forename: Zenaida
authors.initials: Z
authors.lastname: Hernández-Díaz
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: aglez@infomed.sld.cu.
authors.forename: Alina
authors.initials: A
authors.lastname: González-Quevedo
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: isabelfa@infomed.sld.cu.
authors.forename: Isabel
authors.initials: I
authors.lastname: Fernández-Almirall
authors.affiliation: National Genetic Center, Cuba. Electronic address: claudia@cngen.sld.cu.
authors.forename: Claudia
authors.initials: C
authors.lastname: Pérez-López
authors.affiliation: National Genetic Center, Cuba. Electronic address: yaisa@cngen.sld.cu.
authors.forename: Yaisa
authors.initials: Y
authors.lastname: Castillo-Casañas
authors.affiliation: National Genetic Center, Cuba.
authors.forename: Olivia
authors.initials: O
authors.lastname: Martínez-Bonne
authors.affiliation: National Genetic Center, Cuba. Electronic address: amelia@cngen.sld.cu.
authors.forename: Amelia
authors.initials: A
authors.lastname: Cabrera-Rivero
authors.affiliation: National Genetic Center, Cuba. Electronic address: leyenis@cngen.sld.cu.
authors.forename: Leyenis
authors.initials: L
authors.lastname: Valdés-Ramos
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: rosaguerra@inn.sld.cu.
authors.forename: Rosa
authors.initials: R
authors.lastname: Guerra-Badía
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: rebeca@infomed.sld.cu.
authors.forename: Rebeca
authors.initials: R
authors.lastname: Fernández-Carriera
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: cary@infomed.sld.cu.
authors.forename: María Caridad
authors.initials: MC
authors.lastname: Menéndez-Sainz
authors.affiliation: Institute of Neurology and Neurosurgery, Habana, Cuba. Electronic address: sergiogg@infomed.sld.cu.
authors.forename: Sergio
authors.initials: S
authors.lastname: González-García
mesh_terms: D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D000975:Antioxidants / Q000378:metabolism; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002374:Catalase / Q000097:blood; D002421:Caudate Nucleus / Q000378:metabolism / Q000473:pathology; D005260:Female; D005978:Glutathione / Q000097:blood; D005979:Glutathione Peroxidase / Q000097:blood; D005980:Glutathione Reductase / Q000097:blood; D017127:Glycation End Products, Advanced / Q000097:blood; D006801:Humans; D006816:Huntington Disease / Q000097:blood* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D008315:Malondialdehyde / Q000097:blood; D008875:Middle Aged; D010084:Oxidation-Reduction; D018384:Oxidative Stress; D013438:Sulfhydryl Compounds / Q000097:blood; D013482:Superoxide Dismutase / Q000097:blood; D014057:Tomography, X-Ray Computed
chemical_list: D000975:Antioxidants; D015415:Biomarkers; D017127:Glycation End Products, Advanced; D013438:Sulfhydryl Compounds; D008315:Malondialdehyde; D002374:Catalase; D005979:Glutathione Peroxidase; D013482:Superoxide Dismutase; D005980:Glutathione Reductase; D005978:Glutathione
keywords: Advanced oxidative protein products; Age at symptom onset; Caudate atrophy; Huntington's disease; Motor scale; Oxidative stress; Reduced glutathione; Reductase glutathione