On the defect of synthesis ceruloplasmin in the liver polyribosomes in Wilson's disease
β Scribed by V. S. Gaitskhoki; O. I. Kisselev; K. A. Moshkov; L. V. Puchkova; M. M. Shavlovski; V. S. Shulman; V. G. Vacharlovski; S. A. Neifakh
- Publisher
- Springer
- Year
- 1975
- Tongue
- English
- Weight
- 883 KB
- Volume
- 13
- Category
- Article
- ISSN
- 0006-2928
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β¦ Synopsis
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 I s 25-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.
π SIMILAR VOLUMES
The normal human neonate has a copper profile indistinguishable from Wilson's disease, and we have previously postulated that this disease is caused by genetic failure to switch from the fetal to adult mode of copper metabolism. This study validates the developing guinea pig as a suitable animal in
## Abstract Ceruloplasmin is a major ferroxidase in sera, and people with hypoceruloplasminemia might have iron load complications. We used histochemical and Xβray microanalysis techniques on pretreatment liver specimens from five patients with Wilson's disease. Serum levels of ceruloplasmin and fe
## Abstract Wilson's disease (WD) is an inherited autosomalβrecessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's