Cytosolic Cu-Binding Components and the Brindled Mouse Defect
The components that Cu binds to when it first enters cells are likely to have important functions in Cu-metabolism. Menkes disease is an inborn error of Cu-metabolism which apparently involves low activity of an intracellular factor. Thus, studies with the brindled mouse model of Menkes disease may help elucidate the function of an intracellular Cu-binding component. Cu-uptake and the intracellular distribution of Cu were studied with hepatocytes and fibroblasts from normal and brindled mice to identify Cu-binding components and their functions.