title: Total oxidant-scavenging capacities of plasma from glycogen  storage disease type Ia patients as measured by cyclic  voltammetry, FRAP and luminescence techniques.
abstract: It has been suggested that the very low incidence of atherosclerosis in glycogen storage disease type Ia (GSD Ia) subjects might be attributed to elevated levels of uric acid, one of the potent low molecular- weight antioxidants found in plasma. The present communication describes a use of two analytical methods-cyclic voltammetry and ferric reducing ability of plasma-and also two chemiluminescence methods to evaluate the total oxidant-scavenging capacities (TOSC) of plasma from GSD Ia patients. Our results verified the elevation of TOSC in GSD Ia patients and we propose the inclusion of luminescence and cyclic voltammetry assays as reliable methods for estimating TOSC in a variety of clinical disorders. Our findings with the cyclic voltammetry method add support to the assumption that the elevated uric acid levels might be the main contributor to plasma antioxidant capacity and possible protection against atherosclerosis.
authors.affiliation: Department of Pharmaceutics, School of Pharmacy, Richard and Jean Zarbin Chair in Medical Studies, Hebrew University, Hadassah Faculty of Medicine, Jerusalem, 91120, Israel.
authors.forename: E
authors.initials: E
authors.lastname: Koren
authors.affiliation: Department of Pediatrics, Bikur Cholim General Hospital, Jerusalem, Israel.
authors.forename: J
authors.initials: J
authors.lastname: Lipkin
authors.affiliation: Department of Pediatrics, Bikur Cholim General Hospital, Jerusalem, Israel.
authors.forename: A
authors.initials: A
authors.lastname: Klar
authors.affiliation: Pediatric Endocrinology & Metabolic Unit, Soroka Medical University Center, Beer Sheva, Israel.
authors.forename: E
authors.initials: E
authors.lastname: Hershkovitz
authors.affiliation: Faculty of Dental Medicine, Institute for Dental Sciences, Hebrew University, Hadassah Medical Center, Jerusalem, Israel.
authors.forename: I
authors.initials: I
authors.lastname: Ginsburg
authors.affiliation: Department of Pharmaceutics, School of Pharmacy, Richard and Jean Zarbin Chair in Medical Studies, Hebrew University, Hadassah Faculty of Medicine, Jerusalem, 91120, Israel. ronk@ekmd.huji.ac.il.
authors.forename: R
authors.initials: R
authors.lastname: Kohen
mesh_terms: D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004553:Electric Conductivity; D004563:Electrochemistry / Q000379:methods; D036681:Fluorescence Recovery After Photobleaching; D016166:Free Radical Scavengers / Q000032:analysis / Q000097:blood*; D005953:Glycogen Storage Disease Type I / Q000097:blood* / Q000175:diagnosis; D006801:Humans; D008163:Luminescent Measurements / Q000379:methods*; D008165:Luminol / Q000737:chemistry; D016877:Oxidants / Q000032:analysis / Q000097:blood*; D010084:Oxidation-Reduction; D015203:Reproducibility of Results; D055815:Young Adult
chemical_list: D016166:Free Radical Scavengers; D016877:Oxidants; D008165:Luminol