[Interaction of molecular forms of ceruloplasmin with specific membrane receptors from healthy persons and patients with hepatolenticular degeneration]

Abstract

The ceruloplasmin (CP) interaction with the specific receptor localized on the erythrocyte membrane of healthy donors and of patients with hepatolenticular degeneration (HLD) was studied by using Scatchard analysis. It was found that in patients with the Wilson's disease the CP receptor is unaffected by the pathological process. The molecular mass of the erythrocyte receptor of CP is about 130 kDa. The CP binding to the specific receptor is followed by limited proteolysis of the latter. No CP binding to the receptor occurs in the presence of serine protease inhibitors. The CP-like protein previously detected in the sera of Wilson gene carriers can specifically interact with the CP receptor.