Copper and Disease

  • Maria C. Linder
Part of the Biochemistry of the Elements book series (BOTE, volume 10)


Wilson’s disease (hepatolenticular degeneration) was first characterized by S. A. Kinnear Wilson in 1912 (Wilson, 1912). It is an inherited, progressive, and, if untreated, ultimately fatal disease of copper accumulation in the human body, particularly in the liver, brain, and kidney (see Table 9-1). Indeed, the demonstration of excess copper in the liver is a requisite for the diagnosis of Wilson’s disease. The disease is usually seen as hepatic dysfunction in early adolescence, although it has been found in patients as young as four years. Symptoms are nonspecific, commonly with degenerative changes in the brain and cirrhosis of the liver. The incidence of the disease is high among Arabs, Japanese, Chinese, Indians, and Jews (Beam, 1960). The gene for Wilson’s disease appears to be located on chromosome 13 (Frydman et al., 1985).


Copper Deficiency Serum Copper Lysyl Oxidase Copper Metabolism Excess Copper 
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Copyright information

© Springer Science+Business Media New York 1991

Authors and Affiliations

  • Maria C. Linder
    • 1
  1. 1.Department of Chemistry and BiochemistryCalifornia State UniversityFullertonUSA

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