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

Autor: Gaitskhoki, V. S., Kisselev, O. I., Moshkov, K. A., Puchkova, L. V., Shavlovski, M. M., Shulman, V. S., Vacharlovski, V. G., Neifakh, S. A.
Zdroj: Biochemical Genetics; October 1975, Vol. 13 p533-550, 18p
Abstrakt: 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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