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Science 3 December 1999: Vol. 286. no. 5446, pp. 1882 - 1888 DOI: 10.1126/science.286.5446.1882
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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
Read the Full Text
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- R. T. Youker, P. Walsh, T. Beilharz, T. Lithgow, and J. L. Brodsky (2004)
Mol. Biol. Cell
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- Role of Vma21p in Assembly and Transport of the Yeast Vacuolar ATPase.
- P. Malkus, L. A. Graham, T. H. Stevens, and R. Schekman (2004)
Mol. Biol. Cell
15, 5075-5091
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- EDEM Contributes to Maintenance of Protein Folding Efficiency and Secretory Capacity.
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J. Biol. Chem.
279, 44600-44605
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- Multiple sequences in the C terminus of MaxiK channels are involved in expression, movement to the cell surface, and apical localization.
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PNAS
101, 15237-15242
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- Membrane biogenesis and the unfolded protein response.
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J. Cell Biol.
167, 23-25
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- Insufficient Folding of Type IV Collagen and Formation of Abnormal Basement Membrane-like Structure in Embryoid Bodies Derived from Hsp47-Null Embryonic Stem Cells.
- Y. Matsuoka, H. Kubota, E. Adachi, N. Nagai, T. Marutani, N. Hosokawa, and K. Nagata (2004)
Mol. Biol. Cell
15, 4467-4475
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- Hepatic secretion of small lipoprotein particles in apobec-1-/- mice is regulated by the LDL receptor.
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J. Lipid Res.
45, 1649-1659
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- Transport of Meprin Subunits through the Secretory Pathway: ROLE OF THE TRANSMEMBRANE AND CYTOPLASMIC DOMAINS AND OLIGOMERIZATION.
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