Identification of apo- and holo-forms of ceruloplasmin in patients with Wilson's disease using native polyacrylamide gel electrophoresis.
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.
Hirano
Kazuya
K
Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka 569-8686, Japan. ped027@poh.osaka-med.ac.jp
Ogihara
Tohru
T
Ogihara
Hiromi
H
Hiroi
Mayo
M
Hasegawa
Masashi
M
Tamai
Hiroshi
H
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
D001059:Apoproteins; D002570:Ceruloplasmin