title: Identification of apo- and holo-forms of ceruloplasmin in patients with Wilson's disease using native polyacrylamide gel electrophoresis.
abstract: OBJECTIVE
  To determine the serum ratio of holo-ceruloplasmin to total ceruloplasmin in patients with Wilson's disease (WD), we identified apo- and holo-forms of serum ceruloplasmin with native-PAGE electrophoresis.
  
  
  METHODS
  Serum obtained from nine patients with Wilson's disease was subjected to native-PAGE analysis. We also determined the ceruloplasmin level and ferroxidase activity in all of the samples.
  
  
  RESULTS
  Among the nine patients, three had both forms of ceruloplasmin and six had only apo-ceruloplasmin. In the patients who had only apo-ceruloplasmin, the serum ceruloplasmin level was significantly lower than in the patients with both forms.
  
  
  CONCLUSIONS
  Differences in the ability to incorporate copper into ceruloplasmin may have a role in ceruloplasmin concentrations in patients with Wilson's disease.
authors.affiliation: Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka 569-8686, Japan. ped027@poh.osaka-med.ac.jp
authors.forename: Kazuya
authors.initials: K
authors.lastname: Hirano
authors.forename: Tohru
authors.initials: T
authors.lastname: Ogihara
authors.forename: Hiromi
authors.initials: H
authors.lastname: Ogihara
authors.forename: Mayo
authors.initials: M
authors.lastname: Hiroi
authors.forename: Masashi
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tamai
mesh_terms: D000293:Adolescent; D000328:Adult; D001059:Apoproteins / Q000032:analysis*; D002570:Ceruloplasmin / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D004591:Electrophoresis, Polyacrylamide Gel; D005312:Fetal Blood / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000378:metabolism*; D006801:Humans
chemical_list: D001059:Apoproteins; D002570:Ceruloplasmin