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 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.
D000596:Amino Acids / Q000378:metabolism; D000818:Animals; D000918:Antibody Specificity; D002474:Cell-Free System; D002570:Ceruloplasmin / Q000096:biosynthesis* / Q000276:immunology; D006527:Hepatolenticular Degeneration / Q000276:immunology / Q000378:metabolism*; D006720:Homozygote; D006801:Humans; D008099:Liver / Q000378:metabolism*; D011132:Polyribosomes / Q000378:metabolism*; D011817:Rabbits
D000596:Amino Acids; D002570:Ceruloplasmin