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目前国内外对肝豆状核变性(HLD)患者体内是否存在锌代谢异常有不同报道.本文系统地研究了HLD患者和杂合子离体培养皮肤成纤维细胞胞浆内的锌/蛋白比值、胞浆内锌在胞浆内不同分子量蛋白上的分布,以及不同浓度铜孵育对胞浆内锌在不同分子量蛋白上分布的影响,结果发现患者胞浆内锌/蛋白比值虽无显著性变化,但锌在胞浆中不同分子量蛋白上的分布有改变,主要表现在胞浆内金属硫蛋白的锌结合量增高,但在铜孵育早期即从原来的较高水平迅速下降,故推测HLD中的锌分布异常可能是铜代谢异常的继发改变。
At present, there are different reports on the existence of abnormal zinc metabolism in patients with hepatolenticular degeneration (HLD) at home and abroad. This article systematically studied the HLD patients and heterozygous in vitro cultured skin fibroblasts in the cytoplasm of zinc / protein ratio, intracytoplasmic zinc in the cytoplasm of different molecular weight protein distribution, and different concentrations of copper incubation on intracytoplasmic Zinc in different molecular weight protein distribution, the results found that patients with intracytoplasmic zinc / protein ratio although no significant change, but zinc in the cytoplasm of different molecular weight protein distribution has changed, mainly in the cytoplasmic metallothionein Of the zinc binding increased, but decreased rapidly from its original high level in the early stage of copper incubation. Therefore, it is speculated that abnormal zinc distribution in HLD may be a secondary change of copper metabolism abnormality.