Aggresomes and Russell bodies - Symptoms of cellular indigestion?

Ron R. Kopito, Roberta Sitia

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)225-231
Number of pages7
JournalEMBO Reports
Volume1
Issue number3
Publication statusPublished - 2000

ASJC Scopus subject areas

  • Genetics
  • Cell Biology

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