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J. Biol. Chem., Vol. 277, Issue 14, 11709-11714, April 5, 2002
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From the Department of Biological Sciences, Stanford University,
Stanford, California 94305-5020
Endoplasmic reticulum-associated
degradation of misfolded cystic fibrosis transmembrane conductance
regulator (CFTR) protein is known to involve the ubiquitin-proteasome
system. In addition, an ATP-independent proteolytic system has been
suggested to operate in parallel with this pathway and become
up-regulated when proteasomes are inhibited (Jensen, T. J., Loo,
M. A., Pind, S., Williams, D. B., Goldberg, A. L., and
Riordan, J. R. (1995) Cell 83, 129-135). In this
study, we use two independent techniques, pulse-chase labeling and a
noninvasive fluorescence cell-based assay, to investigate the
proteolytic pathways underlying the degradation of misfolded CFTR. Here
we report that only inhibitors of the proteasome have a significant
effect on preventing the degradation of CFTR, whereas cell-permeable
inhibitors of lysosomal degradation, autophagy, and several classes of
protease had no measurable effect on CFTR degradation, when used either
alone or in combination with the specific proteasome inhibitor
carbobenzoxy-L-leucyl-leucyl-L-leucinal (MG132). Our results suggest that ubiquitin-proteasome-mediated degradation is the dominant pathway for disposal of misfolded CFTR in
mammalian cells and provide new mechanistic insight into endoplasmic
reticulum-associated degradation.
A Principal Role for the Proteasome in Endoplasmic
Reticulum-associated Degradation of Misfolded Intracellular Cystic
Fibrosis Transmembrane Conductance Regulator*
,
*
This work was supported in part by National Institutes of
Health Grant R01 DK43994 (to R. R. K.).The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
Supported in part by a postdoctoral training grant from the
National Institutes of Health.
§
To whom correspondence should be addressed: Dept. of Biological
Sciences, Stanford University, 371 Serra Mall, Stanford, CA 94305-5020. Tel.: 650-723-7581; Fax: 650-723-8475; E-mail:
kopito@stanford.edu.
Copyright © 2002 by The American Society for Biochemistry and Molecular Biology, Inc.
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