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On the defect of synthesis ceruloplasmin in the liver polyribosomes in Wilson's disease

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Abstract

Comparative immunochemical analysis of ceruloplasmin-synthesizing polyribosomes in liver biopsies from control subjects and homozygous carriers of the Wilson's mutation was performed. According to I125-antibody binding data, the amount of ceruloplasmin-forming liver polysomes in patients with Wilson's disease was 10–20 times lower than that in non-Wilson patients. Correspondingly, the pulse labeling of ceruloplasmin polypeptides was decreased severalfold in the cell-free liver preparations from patients with Wilson's disease.

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The work was partly supported by the World Health Organization (Agreement No. G3/181/38).

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Gaitskhoki, V.S., Kisselev, O.I., Moshkov, K.A. et al. On the defect of synthesis ceruloplasmin in the liver polyribosomes in Wilson's disease. Biochem Genet 13, 533–550 (1975). https://doi.org/10.1007/BF00484913

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  • DOI: https://doi.org/10.1007/BF00484913

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