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

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 W...

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Bibliographic Details
Published in:Biochemical genetics 1975-10, Vol.13 (9-10), p.533-550
Main Authors: Gaitskhoki, V S, Kisselev, O I, Moshkov, K A, Puchkova, L V, Shavlovski, M M, Shulman, V S, Vacharlovski, V G, Neifakh, S A
Format: Article
Language:English
Subjects:
Amino Acids - metabolism
Animals
Antibody Specificity
Cell-Free System
Ceruloplasmin - biosynthesis
Ceruloplasmin - immunology
Hepatolenticular Degeneration - immunology
Hepatolenticular Degeneration - metabolism
Homozygote
Humans
Liver - metabolism
Polyribosomes - metabolism
Rabbits
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Summary: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 several-fold in the cell-free liver preparations from patients with Wilson's disease.
ISSN:0006-2928
1573-4927
DOI:10.1007/BF00484913