Aggresomes and Russell bodies - Symptoms of cellular indigestion?

被引:199
作者
Kopito, RR
Sitia, R [1 ]
机构
[1] HSR, DIBIT, Dept Mol Pathol & Med, I-20132 Milan, Italy
[2] Stanford Univ, Dept Biol Sci, Stanford, CA 94305 USA
关键词
D O I
10.1093/embo-reports/kvd052
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
All cells are equipped with a proteolytic apparatus that eliminates damaged, misfolded and incorrectly assembled proteins. The principal engine of cytoplasmic proteolysis, the 26S proteasome, requires that substrates be unfolded to gain access to the active site; consequently, it is relatively ineffective at degrading aggregated proteins. Cellular indigestion occurs when the production of aggregation-prone proteins exceeds the cell's (or organelle's) capacity to eliminate them. Cellular pathways that resolve this indigestion exist, but appear to have limited capacities. Russell bodies and aggresomes are manifestations of cellular indigestion in the endoplasmic reticulum and cytoplasmic compartments, respectively, and are often associated with disease.
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收藏
页码:225 / 231
页数:7
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