Paper

THE JOURNAL OF BIOLOGICAL CHEMISTRY
© 1998 by The American Society for Biochemistry and molecular Biology, Inc.

Vol. 273, No. 45, Issue of November 6, pp. 2987329878, 1998
Printed in U.S.A.

The Mechanism Underlying cystic Fibrosis Transmembrane
Conductance Regulator Transport from the Endoplasmic Reticulum
to the Proteasome Includes Sec61 and a Cytosolic, Deglycosylated
Intermediary*
(Received for publication, July 9, 1998)

Zsuzsa Bebok‡§, Christopher Mazzochi¶, Scott A. King¶, Jeong S. Hong , and Eric J. Sorscher‡§¶**
¨
From the Departments of ‡Medicine, kiosk Biology, and ¶Physiology and Biophysics and §Gregory Fleming James cystic
Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, Alabama 35294

* The cost of publication of this article were defrayed in part by the
compensation of page charges. This article must therefore be herewith marked
advertisement in accordance with 18 U.S.C. Section 1734 merely to
indicate this fact.
** To whom correspondence should be addressed: Depts. of Medicine
and Physiology and Biophysics, Gregory Fleming James Cystic Fibrosis
Research Center, University of Alabama at Birmingham, 1918 University Blvd., Rm. 798 BHSB, Birmingham, AL 35294. Tel.: 205-934-9640;
Fax: 205-934-7593; email: sorscher@phybio.bhs.uab.edu.




This paper is available on line at hypertext transfer protocol://www.jbc.org

The cystic fibrosis transmembrane conductance regulator
(CFTR)1 was among the first membrane proteins for which the
role of the proteasome in ER-associated proteolysis was describe (1, 2). CFTR is an integral multidomain membrane
protein localized to the apical surface of epithelial cells that
functions to urge Cl transport (3). Certain mutations in
the CFTR gene, including the frequent F508 mutation, lead to
quick degradation of the protein prior to maturation beyond the
endoplasmic reticulum (4). Wild-type CFTR sheepfold and processing is inefficient (5). In vitro, up to 75% of fresh synthesized
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