title: Hereditary ceruloplasmin deficiency increases advanced glycation end products in the brain.
abstract: We investigated the role of ceruloplasmin in the antioxidative process in the brain in a patient with hereditary ceruloplasmin deficiency (HCD). Immunohistochemistry revealed an accumulation of Nepsilon-(carboxymethyl) lysine (CML) in basal ganglia of the HCD brain. In vitro study disclosed that ceruloplasmin inhibited CML formation from glycated proteins through the reaction of Fe2+ with H2O2 by Fenton reaction. These data suggest that ceruloplasmin plays an important role in the protection of neurons against oxidative stress associated with iron metabolism.
authors.affiliation: Third Department of Internal Medicine, Yamagata University School of Medicine, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Tajima
authors.forename: T
authors.initials: T
authors.lastname: Kawanami
authors.forename: R
authors.initials: R
authors.lastname: Nagai
authors.forename: S
authors.initials: S
authors.lastname: Horiuchi
authors.forename: T
authors.initials: T
authors.lastname: Kato
mesh_terms: D001921:Brain / Q000473:pathology*; D002570:Ceruloplasmin / Q000172:deficiency* / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008664:Metal Metabolism, Inborn Errors / Q000235:genetics* / Q000473:pathology; D008875:Middle Aged
chemical_list: D002570:Ceruloplasmin