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PMID: 11256604 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Review

Aggresomes and Russell bodies. Symptoms of cellular indigestion?

EMBO reports ·Vol. 1 ·No. 3 ·2000-09-00 ·Pages 225-31

Kopito RR, Sitia R

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.

MeSH Terms
Cytoplasm/metabolism,pathology,ultrastructure Cytoplasmic Granules/metabolism,pathology,ultrastructure Disulfides/metabolism Endoplasmic Reticulum/metabolism,pathology,ultrastructure Humans Peptide Hydrolases/metabolism Proteasome Endopeptidase Complex Protein Denaturation Protein Folding Proteins/chemistry,metabolism Ubiquitins/metabolism
Chemicals
Disulfides Proteins Ubiquitins Peptide Hydrolases Proteasome Endopeptidase Complex ATP dependent 26S protease
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Kopito R R
Department of Biological Sciences, Stanford University, CA 94305-5020, USA.
Sitia R
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Article Info
Journal
EMBO reports
Abbr.
EMBO Rep
ISSN
1469-221X
DOI
10.1093/embo-reports/kvd052
Published
2000-09-00
Pages
225-31
Language
English
Region
England
NLM ID
100963049
PMCID
PMC1083726
Subset
IM
External Links
PubMed source DOI: 10.1093/embo-reports/kvd052 PMC Full Text View journal details
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