Study of level and functions of P-type ATPase7B in hepatic cells of patients with hepatolenticular degeneration
- VernacularTitle:肝豆状核变性患者肝细胞中P型ATP7B酶水平及功能的研究
- Author:
Daping LV
;
Yongzhu HAN
;
Xun WANG
- Publication Type:Journal Article
- Keywords:
hepatolenticular degeneration;
hepatic cell;
P-type ATPase7B
- From:
Journal of Clinical Neurology
1993;0(03):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the level and main functions of P-type ATPase7B in hepatic cells of patients with hepatolenticular degeneration(HLD).Methods The hepatic cells from 5 normal controls and 9 patients with HLD were cultured in vitro. P-type ATPase7B levels in hepatic cells were examined and compared by SDS-PAGE and Western-blot techniques.Results Compared with the controls, 9 patients displayed various changes of electrophoresis strip. Almost normal strips at 155?103 were found in 3 cases, no strip was found in 1 case, and thinner and lighter strips were showed in the remain 5 cases. 6 cases presented abnormal specific reaction strips.Conclusion Mutations of gene ATPase7B in HLD patients cause change of P-type ATPase7B in quantity and quality, thus leads to dysmetabolism of copper.
