Active Copper Transport in Mammalian Tissues—a Possible Role in Wilson's Disease

Abstract

IT has long been known that there are two major circulating fractions of serum copper. In the normal person, 95 per cent of the copper is carried by the alpha₂-globulin copper oxidase, ceruloplasmin, and the remainder was said to be ‘free’ or albumin-bound. A previous report¹ demonstrated a third physiologically important dialysable fraction of serum copper bound to amino-acids which facilitated the transport of copper across a semi-permeable membrane in vitro. The present investigation shows that there is an active transport mechanism for copper which operates in a metabolically active cell system. The findings lend themselves to interesting speculations with regard to the pathogenesis of Wilson's disease.