CYTOCHROME OXIDASE DEFICIENCY IN WILSON'S DISEASE: A SUGGESTED CERULOPLASMIN FUNCTION | Zendy

M. H. K. Shokeir | Zendy; Donald C. Shreffler | Zendy

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CYTOCHROME OXIDASE DEFICIENCY IN WILSON'S DISEASE: A SUGGESTED CERULOPLASMIN FUNCTION

Author(s) -

M. H. K. Shokeir,

Donald C. Shreffler

Publication year - 1969

Publication title -

proceedings of the national academy of sciences

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 5.011

H-Index - 771

eISSN - 1091-6490

pISSN - 0027-8424

DOI - 10.1073/pnas.62.3.867

Subject(s) - ceruloplasmin , cytochrome c oxidase , oxidase test , enzyme , biology , medicine , endocrinology , biochemistry

The hypothesis is advanced that ceruloplasmin functions in enzymatic transfer of copper to copper-containing enzymes, such as cytochrome oxidase. To test this hypothesis, leucocytes from Wilson's disease patients, heterozygous carriers, and normal subjects were assayed for cytochrome-oxidase activity. The data reported here show markedly reduced levels of activity in Wilson's disease cases and moderate reductions in heterozygous individuals relative to normal controls. These observations and a close correlation between the level of cytochrome-oxidase activity in the leucocytes and ceruloplasmin in the serum tend to support the hypothesis.

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