title: Coenzyme Q10 and oxidative imbalance in Down syndrome: biochemical and clinical aspects.
abstract: Down syndrome (DS) is a chromosomal abnormality (trisomy 21) associated with mental retardation and Alzheimer-like dementia, characteristic change of the individual's phenotype and premature ageing. Oxidative stress is known to play a major role in this pathology since a gene dose effect leads to elevated ratio of superoxide dismutase to catalase/glutathione peroxidase compared to controls in all age categories suggesting that oxidative imbalance contributes to the clinical manifestation of DS. Hyperuricemia is another feature of DS that has an interesting relationship with oxidative stress since uric acid represents an important free radical scavenger. However its formation is connected to the conversion of Xanthine dehydrogenase (XDH) to Xanthine oxidase (XO) which leads to concomitant production of free radicals. Here we report that plasma samples from DS patients in pediatric age, despite an increased total antioxidant capacity, largely due to elevated Uric acid content (UA), present significantly elevated markers of oxidative damage such as increased allantoin levels. Moreover DS plasma samples do not differ from healthy control ones in terms of Coenzyme Q10 and susceptibility to peroxidative stimuli. On the contrary, lymphocyte and platelet CoQ10 content was significantly lower in DS patients, a fact that might underlie oxidative imbalance at a cellular level.
authors.affiliation: Institute of Biochemistry, Polytechnic University of the Marche, Ancona, Italy. luca.tiano@unicam.it
authors.forename: L
authors.initials: L
authors.lastname: Tiano
authors.forename: L
authors.initials: L
authors.lastname: Padella
authors.forename: P
authors.initials: P
authors.lastname: Carnevali
authors.forename: O
authors.initials: O
authors.lastname: Gabrielli
authors.forename: F
authors.initials: F
authors.lastname: Bruge
authors.forename: F
authors.initials: F
authors.lastname: Principi
authors.forename: G P
authors.initials: GP
authors.lastname: Littarru
mesh_terms: D000481:Allantoin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000378:metabolism*; D006801:Humans; D018384:Oxidative Stress / Q000187:drug effects*; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000378:metabolism; D014527:Uric Acid / Q000097:blood
chemical_list: D014451:Ubiquinone; D014527:Uric Acid; D000481:Allantoin; C024989:coenzyme Q10