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Review
. 2009 Mar 26;458(7237):453-60.
doi: 10.1038/nature07962.

The ubiquitylation machinery of the endoplasmic reticulum

Christian Hirsch  1 , Robert Gauss, Sabine C Horn, Oliver Neuber, Thomas Sommer
Affiliations
Review

The ubiquitylation machinery of the endoplasmic reticulum

Christian Hirsch et al. Nature. .

Abstract

As proteins travel through the endoplasmic reticulum (ER), a quality-control system retains newly synthesized polypeptides and supports their maturation. Only properly folded proteins are released to their designated destinations. Proteins that cannot mature are left to accumulate, impairing the function of the ER. To maintain homeostasis, the protein-quality-control system singles out aberrant polypeptides and delivers them to the cytosol, where they are destroyed by the proteasome. The importance of this pathway is evident from the growing list of pathologies associated with quality-control defects in the ER.

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