Cytosolic Cu-Binding Components and the Brindled Mouse Defect

  • F. Palida
  • G. Waldrop
  • P. Lonergan
  • M. Ettinger

Abstract

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.

Keywords

Electrophoresis Cytosol Lysyl 

References

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    Beratis, N.G., Price, P., LaBadie, G. and Hirschhorn, K., Pediat. Res. 12, 699–702 (1978).PubMedCrossRefGoogle Scholar
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    Royce, P.M., Camakaris, J. and Danks, D., Biochem. J. 192, 579–586 (1980).PubMedGoogle Scholar

Copyright information

© Plenum Press, New York 1988

Authors and Affiliations

  • F. Palida
    • 1
  • G. Waldrop
    • 1
  • P. Lonergan
    • 1
  • M. Ettinger
    • 1
  1. 1.Department of BiochemistryState University of New York at BuffaloBuffaloUSA

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