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Science 3 December 1999:
Vol. 286. no. 5446, pp. 1882 - 1888
DOI: 10.1126/science.286.5446.1882

Review

Setting the Standards: Quality Control in the Secretory Pathway

Lars Ellgaard, Maurizio Molinari, Ari Helenius *

A variety of quality control mechanisms operate in the endoplasmic reticulum and in downstream compartments of the secretory pathway to ensure the fidelity and regulation of protein expression during cell life and differentiation. As a rule, only proteins that pass a stringent selection process are transported to their target organelles and compartments. If proper maturation fails, the aberrant products are degraded. Quality control improves folding efficiency by retaining proteins in the special folding environment of the endoplasmic reticulum, and it prevents harmful effects that could be caused by the deployment of incompletely folded or assembled proteins.

Institute of Biochemistry, Swiss Federal Institute of Technology (ETH), Universitätstrasse 16, CH-8092 Zürich, Switzerland.
*   To whom correspondence should be addressed. E-mail: ari.helenius{at}bc.biol.ethz.ch


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Transport of Meprin Subunits through the Secretory Pathway: ROLE OF THE TRANSMEMBRANE AND CYTOPLASMIC DOMAINS AND OLIGOMERIZATION.
J. A. Hengst and J. S. Bond (2004)
J. Biol. Chem. 279, 34856-34864