Tissue Copper Proteins in Wilson's Disease

Abnormalities in copper metabolism are a consistent feature of Wilson's disease,¹and it has been demonstrated that these abnormalities antedate both pathological lesions and the onset of clinical illness²and can be detected in infancy.³The occurrence of elevated tissue copper early in life relative to the onset of symptoms²,⁴and the improvement which may follow treatment with copper mobilizing agents⁵,⁶suggest that accumulation of copper in the tissues is the primary factor in the production of the characteristic deficits in neurological, hepatic, and renal function. Among the manifestations of this abnormal copper metabolism in Wilson's disease are two which are similar to the condition of the normal newborn infant; first, a reduced amount of normal plasma ceruloplasmin,⁷and, second, a greatly elevated concentration of copper in the liver.⁸,⁹One phase of the present investigation deals with the possible relationship between the type