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Vol. 13, Issue 6, 1806-1818, June 2002
and
*Institut für Biochemie, Universität Stuttgart,
70569 Stuttgart, Germany; and Protein quality control is an essential function of the endoplasmic
reticulum. Misfolded proteins unable to acquire their native
conformation are retained in the endoplasmic reticulum, retro-translocated back into the cytosol, and degraded via the ubiquitin-proteasome system. We show that efficient degradation of
soluble malfolded proteins in yeast requires a fully competent early
secretory pathway. Mutations in proteins essential for ER-Golgi protein
traffic severely inhibit ER degradation of the model substrate CPY*. We
found ER localization of CPY* in WT cells, but no other specific
organelle for ER degradation could be identified by electron microscopy
studies. Because CPY* is degraded in COPI coat mutants, only a minor
fraction of CPY* or of a proteinaceous factor required for degradation
seems to enter the recycling pathway between ER and Golgi. Therefore,
we propose that the disorganized structure of the ER and/or the
mislocalization of Kar2p, observed in early secretory mutants, is
responsible for the reduction in CPY* degradation. Further, we observed
that mutations in proteins directly involved in degradation of
malfolded proteins (Der1p, Der3/Hrd1p, and Hrd3p) lead to morphological
changes of the endoplasmic reticulum and the Golgi, escape of CPY* into
the secretory pathway and a slower maturation rate of wild-type CPY.
Max-Delbrück-Centrum
für Molekulare Medizin, 13125 Berlin, Germany
Corresponding author. E-mail address:
dieter.wolf{at}po.uni-stuttgart.de.
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