An array of mitochondrial alterations in the hepatocytes of long‐evans cinnamon rats
An array of mitochondrial alterations in the hepatocytes of long‐evans cinnamon rats
复制标题
长埃文斯肉桂大鼠肝细胞中的一系列线粒体变化
作者：
I. Sternlieb;N. Quintana;I. Volenberg;M. Schilsky
为了确定铜毒性的细胞靶标，我们研究了 23 只 10 至 89 周龄的 Long-Evans Cinnamon (LEC) 大鼠肝脏中肝细胞的超微结构。肝铜浓度范围为 325 至 2,126（平均 930）μg/g 干重。 13只大鼠在处死时表现出不同程度的黄疸。许多细胞核因细胞质内陷而凹陷。线粒体显示出明显的异常。其中包括明显的多形性；基质密度增加或减少；嵴重新排列、伸长、扩张、堆叠或消失；不存在基质颗粒；存在絮状电子致密沉积物的基质包裹体；和退行性改变。超微结构变化的严重程度与肝铜浓度无关，但与大鼠黄疸程度相关。某些线粒体变化与威尔逊病患者肝细胞中的变化相似，证实这些细胞器是铜毒性的重要目标。 （肝病学 1995；22：1782-1787）。
In an attempt to identify the cellular targets of copper toxicity, we studied the ultrastructure of hepatocytes in the livers of 23 Long‐Evans Cinnamon (LEC) rats ranging in age from 10 to 89 weeks. The hepatic copper concentration ranged from 325 to 2,126 (mean, 930) μg/g dry weight. Thirteen rats displayed varying degrees of jaundice at the time of killing. Numerous nuclei were indented by cytoplasmic invaginations. Conspicuous abnormalities were displayed by mitochondria. These included marked pleomorphism; increased or diminished matrical density; rearrangement, elongation, dilatation, stacking, or disappearance of cristae; absence of matrical granules; presence of matrical inclusions of flocculent electron‐dense deposits; and degenerative changes. The severity of the ultrastructural changes did not correlate with the hepatic copper concentration but did correlate with the degree of the icterus displayed by the rats. Certain mitochondrial changes resembled those encountered in the hepatocytes of patients with Wilson's disease, confirming that these organelles are important targets of copper toxicity. (Hepatology 1995; 22:1782‐1787).
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LEC 突变的多效性效应：威尔逊病的啮齿动物模型。
DOI：
10.1152/ajpgi.1994.266.5.g907
发表时间：
1994
期刊：
The American journal of physiology
影响因子：
--
作者：
Schilsky,ML;Stockert,RJ;Sternlieb,I
通讯作者：
Sternlieb,I
Long-Evans Cinnamon 大鼠中威尔逊病基因的表达存在缺陷。
DOI：
10.1042/bj3010001
发表时间：
1994
期刊：
The Biochemical journal
影响因子：
--
作者：
Yamaguchi,Y;Heiny,ME;Shimizu,N;Aoki,T;Gitlin,JD
通讯作者：
Gitlin,JD
影响因子：
29.4
作者：
SOKOL, RJ;TWEDT, D;NEUSCHWANDERTETRI, BA
通讯作者：
NEUSCHWANDERTETRI, BA
影响因子：
4.1
作者：
Sternlieb,I
通讯作者：
Sternlieb,I