93 physicochemical properties of ceruloplasmin from normal and wilson's disease subjects

ABSTRACT Although most patients with Wilson's disease have low levels of serum ceruloplasmin, an occasional patient presents with a normal level. This variation might be due to

differences in the structure of ceruloplasmin. To investigate this possibility, physico-chemical properties of ceruloplasmin from a Wilson's disease patient with normal levels of

ceruloplasmin, from a second, unrelated, patient with low levels and from normal subjects were compared. Highly purified ceruloplasmin from the patient with normal levels was chromatographed

on hydroxylapatite. The major component obtained had the same electrophoretic mobility (at pH 5.6, 6.4, 8.9), tryptic peptide map, oxidase activity, copper, amino acid and sugar composition

as the major component from normal subjects. The minor hydroxylapatite component from the patient had the same electrophoretic mobility (at pH 5.6), tryptic peptide map and oxidase activity

as the minor component from normal subjects. The major and minor ceruloplasmin components were found to differ from each other by only one peptide spot. Ceruloplasmin from the Wilson's

disease patient with a low serum ceruloplasmin concentration had the same chromatographic and electrophoretic characteristics as normal ceruloplasmin. Thus it appears that ceruloplasmin in

Wilson's disease is structurally normal. Penicillamine administered to the patient with normal ceruloplasmin concentration caused an increased excretion of copper. Concommitantly,

ceruloplasmin activity became undetectable in his plasma and reappeared only after the drug was discontinued. This finding suggests that in Wilson's disease, the sensitivity of the

ceruloplasmin synthesizing system to copper may be diminished. (SPR) ARTICLE PDF AUTHOR INFORMATION Author notes * Neil A Holtzman and Michael A Naughton: By invitation AUTHORS AND

AFFILIATIONS * Johns Hopkins Univ. Sch. of Med., Md., Baltimore Neil A Holtzman, Michael A Naughton & Robert E Cooke Authors * Neil A Holtzman View author publications You can also

search for this author inPubMed Google Scholar * Michael A Naughton View author publications You can also search for this author inPubMed Google Scholar * Robert E Cooke View author

publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Holtzman, N., Naughton, M. &

Cooke, R. 93 Physicochemical Properties of Ceruloplasmin from Normal and Wilson's Disease Subjects. _Pediatr Res_ 1, 224 (1967). https://doi.org/10.1203/00006450-196705000-00099

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