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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Veröffentlicht in:	Biochemical genetics 1975-10, Vol.13 (9-10), p.533-550
Hauptverfasser:	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:	Artikel
Sprache:	eng
Schlagworte:	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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Zusammenfassung:	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