An international symposium on Wilson's and Menkes' diseases.
An international symposium on Wilson's and Menkes' diseases.
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关于威尔逊病和门克斯病的国际研讨会。
DOI：
10.1002/hep.510240433
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发表时间：
1996
期刊：
影响因子：
--
通讯作者：
Sternlieb,I
中科院分区：
文献类型：
--
作者：
Ferenci,P;Gilliam,TC;Gitlin,JD;Packman,S;Schilsky,ML;Sokol,RJ;Sternlieb,I
COPPER TOXICOSIS enriched in canalicular plasma membranes obtained from the livers of Groningen-Yellow rats showed ATP-dependent M. Suzuki (Toho University, Tokyo, Japan) summarized copper transport which conformed to Michaelis-Menten ki-data regarding copper metabolism in LEC rats, the best aninetics (Km 8.6 mmol/L and Vmax 6.9 nmol/min/mg protein). A mal model for WD. This mutation is characterized by a deficomparison of 64copper uptake by basolateral and canalicular ciency of oxidase active ceruloplasmin in the serum, marked membrane vesicles showed less isotope in the former, indicat-increases in hepatic copper concentration, and reduced biliing that the rat hepatocellular plasma membrane accomo-ary copper excretion. 2 Steady state levels of ceruloplasmin, dates ATP-dependent copper transporters in both domains. messenger RNA (mRNA) in the livers of LEC rats and in Similar results were observed when measurements of 64cop-control Long-Evans Agouti rats were similar, as were the per transport were made using vesicles obtained from human amounts of newly synthesized ceruloplasmin peptide. Belivers. Unfortunately the lack of a satisfactory antibody to cause transcription and translation of ceruloplasmin mRNA the Wilson’s disease (WD) gene product (ATP7B) is delaying seem to proceed normally, the data suggest a defect in the the cellular localization, thereby leaving the canalicular incorporation of copper into the apoprotein. membrane, the lysosomes, and the endoplasmic reticulum as Using Northern blot analysis, Yamaguchi et al.(St. Louis, the most likely candidate sites. MO and Tokyo, Japan) detected an abundance of the WD The same Dutch group found that orally ingested and intra-gene homologue mRNA in the livers of controls, but not in venously administered copper were processed differently by those of LEC rats, in accord with earlier reports of tissue hepatocytes. Dietary copper proceeded in part via a GSH-specific expression of the WD gene in humans. This finding is consistent with the results of Wu et al. who defined the
