Ceruloplasmin in Wilson's Disease

Neil A. Holtzman; Michael A. Naughton; Frank L. Iber; Bonnie M. Gaumnitz

doi:10.1172/JCI105606

^{Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Biophysics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{The Joseph P. Kennedy Memorial Laboratories, Children's Medical and Surgical Center, The Johns Hopkins Hospital, Baltimore, Md.}

†

Address requests for reprints to Dr. Neil A. Holtzman, Dept. of Pediatrics, The Johns Hopkins Hospital, Baltimore, Md. 21205.

Submitted for publication October 21, 1966; accepted March 6, 1967.

This work was supported by U. S. Public Health Service basic science training grant GM-0624 and research grant AM-06278 AMP.

Find articles by Holtzman, N. in: PubMed | Google Scholar

^{Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Biophysics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{The Joseph P. Kennedy Memorial Laboratories, Children's Medical and Surgical Center, The Johns Hopkins Hospital, Baltimore, Md.}

†

Address requests for reprints to Dr. Neil A. Holtzman, Dept. of Pediatrics, The Johns Hopkins Hospital, Baltimore, Md. 21205.

Submitted for publication October 21, 1966; accepted March 6, 1967.

This work was supported by U. S. Public Health Service basic science training grant GM-0624 and research grant AM-06278 AMP.

Find articles by Naughton, M. in: PubMed | Google Scholar

^{Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Biophysics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{The Joseph P. Kennedy Memorial Laboratories, Children's Medical and Surgical Center, The Johns Hopkins Hospital, Baltimore, Md.}

†

Address requests for reprints to Dr. Neil A. Holtzman, Dept. of Pediatrics, The Johns Hopkins Hospital, Baltimore, Md. 21205.

Submitted for publication October 21, 1966; accepted March 6, 1967.

This work was supported by U. S. Public Health Service basic science training grant GM-0624 and research grant AM-06278 AMP.

Find articles by Iber, F. in: PubMed | Google Scholar

^{Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Biophysics, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Md.}

^{The Joseph P. Kennedy Memorial Laboratories, Children's Medical and Surgical Center, The Johns Hopkins Hospital, Baltimore, Md.}

†

Address requests for reprints to Dr. Neil A. Holtzman, Dept. of Pediatrics, The Johns Hopkins Hospital, Baltimore, Md. 21205.

Submitted for publication October 21, 1966; accepted March 6, 1967.

This work was supported by U. S. Public Health Service basic science training grant GM-0624 and research grant AM-06278 AMP.

Find articles by Gaumnitz, B. in: PubMed | Google Scholar

Ceruloplasmin was highly purified from one patient with Wilson's disease and partially purified from a second unrelated patient. The highly purified ceruloplasmin was indistinguishable from normal ceruloplasmin by electrophoresis, tryptic peptide map, oxidase activity, and copper, amino acid, and sugar composition. The partially purified ceruloplasmin was indistinguishable electrophoretically from normal ceruloplasmin. With penicillamine therapy, ceruloplasmin disappeared from the serum of the first patient; it reappeared after the drug was discontinued. The significance of this observation in regard to the basic defect in Wilson's disease is discussed.

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