Quality control in the endoplasmic reticulum

L Ellgaard, A Helenius - Nature reviews Molecular cell biology, 2003 - nature.com
L Ellgaard, A Helenius
Nature reviews Molecular cell biology, 2003nature.com
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 …
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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