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  • Title: Quality control in the endoplasmic reticulum.
    Author: Ellgaard L, Helenius A.
    Journal: Nat Rev Mol Cell Biol; 2003 Mar; 4(3):181-91. PubMed ID: 12612637.
    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.
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