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Nat Rev Mol Cell Biol. 2003 Mar;4(3):181-91.

Quality control in the endoplasmic reticulum.

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1
Institute of Biochemistry, Swiss Federal Institute of Technology (ETH) Zürich, Hönggerberg, CH - 8093 Zürich, Switzerland.

Abstract

The endoplasmic reticulum (ER) has a quality-control system for 'proof-reading' newly synthesized proteins, so that only native conformers reach their final destinations. Non-native conformers and incompletely assembled oligomers are retained, and, if misfolded persistently, they are degraded. As a large fraction of ER-synthesized proteins fail to fold and mature properly, ER quality control is important for the fidelity of cellular functions. Here, we discuss recent progress in understanding the conformation-specific sorting of proteins at the level of ER retention and export.

PMID:
12612637
DOI:
10.1038/nrm1052
[Indexed for MEDLINE]
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