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Abnormal accumulation of apo-ceruloplasmin in a rat model of Wilson's disease

1993; Elsevier BV; Volume: 1; Issue: 4 Linguagem: Inglês

10.1016/0928-4346(93)90045-h

1879-1182

N KOJIMAHARAA, H NAKABAYASHITADAOIKEDAMICHIOMORI, Toshiko Ikeda, Makoto Mori, Toshio Shikata, Mariko Esumi,

Trace Elements in Health

We examined the mechanism of decrease in serum ceruloplasmin (Cp) in LEC rats, an animal model of Wilson's disease. Northern blot analysis showed that the level of Cp mRNA in the liver of LEC rats was similar to that in control LEA rats. Moreover the serum level of Cp in LEC rats, determined by ELISA, was 70% of that in LEA rats, but its p-phenylenediamine oxidase activity was significantly decreased. Serum Cp was detected as two bands by polyacrylamide gel electrophoresis and immunoblot analysis. Cp in the band with higher mobility had ferroxidase activity and was the predominant form in LEA rats, whereas that in the band of lower mobility was inactive and was the major form in LEC rats. Dialysis of serum against sodium cyanate to remove copper from Cp resulted in disappearance of the form of Cp of higher mobility with increase in the slower migrating form. These results demonstrate that the faster and slower migrating forms of Cp are a holo-Cp and apo-Cp, respectively, and that LEC rats accumulate apo-Cp in their serum. A disturbance of copper binding to apo-Cp may be responsible for pathogenesis in LEC rats.