title: Evaluation of the possible role of coenzyme Q10 and vitamin E in juvenile neuronal ceroid-lipofuscinosis (JNCL).
abstract: The juvenile type of neuronal ceroid lipofuscinosis (JNCL) is a recessively inherited, progressive neurodegenerative disease. In this study the levels of the antioxidant factors coenzyme Q10 (CoQ10) and vitamin E (alpha-tocopherol) were measured in plasma samples of 29 JNCL patients and compared to 48 healthy controls. A significant reduction of the coenzyme Q10 level (0.59 +/- 0.25 microgram/ml) was observed in JNCL patients when compared to control subjects (0.80 +/- 0.26 microgram/ml). The level of vitamin E was also reduced markedly in JNCL patients when compared to controls (10.4 +/- 4.1 and 12.1 +/- 4.5 micrograms/ml, respectively). The low levels of CoQ10 and vitamin E in JNCL patients may indicate an impaired antioxidant protection in this disease.
authors.affiliation: Children's Hospital, University of Helsinki, Finland.
authors.forename: T
authors.initials: T
authors.lastname: Westermarck
authors.forename: L
authors.initials: L
authors.lastname: Aberg
authors.forename: P
authors.initials: P
authors.lastname: Santavuori
authors.forename: E
authors.initials: E
authors.lastname: Antila
authors.forename: P
authors.initials: P
authors.lastname: Edlund
authors.forename: F
authors.initials: F
authors.lastname: Atroshi
mesh_terms: D000293:Adolescent; D000328:Adult; D000975:Antioxidants / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D003067:Coenzymes; D006801:Humans; D009472:Neuronal Ceroid-Lipofuscinoses / Q000097:blood*; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood / Q000502:physiology; D014810:Vitamin E / Q000097:blood / Q000502:physiology*
chemical_list: D000975:Antioxidants; D003067:Coenzymes; D014451:Ubiquinone; D014810:Vitamin E; D002784:Cholesterol; C024989:coenzyme Q10