Biopolymers and cell. 1991. Volume 7. № 3. 24 - 31

PUCHKOVA L. V., VERBINA I. A., DENEZHKINA V. V., GAITSKHOKI V. S., NEIFAKH S. A.

Molecular Defect of Biliary Excretion of Copper in Patients with Hepatolcnticular Degeneration

Summary

    The screening of the samples of blood serum of homo- and heterozygotes with Wilson's disease gene was using immunoelectrophoretic methods. It was shown that patients with Wilsonian gene alongside with normal ceruloplasmin contain in serum ceruloplasmin-like protein too. The same protein was involved into the process of liver cooper secretion in normal subjects. The molecular defect of biliary excretion of copper in patients with Wilson's disease is discussed.