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Identification of apo- and holo-forms of ceruloplasmin in patients with Wilson's disease using native polyacrylamide gel electrophoresis.

Abstract：

OBJECTIVES: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. DESIGN AND 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.

journal_name

Clin Biochem

journal_title

Clinical biochemistry

authors

Hirano K,Ogihara T,Ogihara H,Hiroi M,Hasegawa M,Tamai H

doi

10.1016/j.clinbiochem.2004.09.008

subject

Has Abstract

pub_date

2005-01-01 00:00:00

pages

9-12

issue

1

eissn

0009-9120

issn

1873-2933

pii

S0009-9120(04)00248-6

journal_volume

38

pub_type

杂志文章

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