Endocrinopathies in the Family of Endoplasmic Reticulum (ER) Storage Diseases: Disorders of Protein Trafficking and the Role of ER Molecular Chaperones*

Paul S. Kim; Peter Arvan

doi:10.1210/edrv.19.2.0327

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Publisher: Oxford University Press
Copyright: Copyright © 1998 by The Endocrine Society
ISSN: 0163-769X
eISSN: 1945-7189
DOI: 10.1210/edrv.19.2.0327
pmid: 9570036
Publisher site: See Article on Publisher Site

Abstract

0163-769X/98/$03.00/0 Endocrine Reviews 19(2): 173–202 Copyright © 1998 by The Endocrine Society Printed in U.S.A. Endocrinopathies in the Family of Endoplasmic Reticulum (ER) Storage Diseases: Disorders of Protein Trafficking and the Role of ER Molecular Chaperones* PAUL S. KIM AND PETER ARVAN Division of Endocrinology (P.S.K.), University of Cincinnati College of Medicine, Cincinnati, Ohio 45267; and Division of Endocrinology (P.A.) and Department of Developmental and Molecular Biology, Albert Einstein College of Medicine, Bronx, New York 10461 I. Introduction I. Introduction A. Overview A. Overview B. Protein folding in the ER C. Supervised folding: the concept of molecular chap- LL EUKARYOTIC cells secrete proteins. Higher eu- A karyotic tissues, in general, and many endocrine erones and folding catalysts glands, in particular, are differentiated to release abundant D. Co- and posttranslational modifications are factors quantities of specialized proteins. Most of the proteins re- that can influence folding leased from cells are carried to the plasma membrane via the II. ER Molecular Chaperones, Folding Catalysts, and Mo- biosynthetic transport pathway. The entire pathway is com- lecular Escorts prised of specific transport vesicles that shuttle their cargo A. Binding protein (BiP) through a series of intracellular way-stations (1): at each B. GRP94 successive

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Endocrine Reviews – Oxford University Press

Published: Apr 1, 1998

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Endocrinopathies in the Family of Endoplasmic Reticulum (ER) Storage Diseases: Disorders of Protein Trafficking and the Role of ER Molecular Chaperones*

Endocrinopathies in the Family of Endoplasmic Reticulum (ER) Storage Diseases: Disorders of Protein Trafficking and the Role of ER Molecular Chaperones*

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Endocrinopathies in the Family of Endoplasmic Reticulum (ER) Storage Diseases: Disorders of Protein Trafficking and the Role of ER Molecular Chaperones*

Endocrinopathies in the Family of Endoplasmic Reticulum (ER) Storage Diseases: Disorders of Protein Trafficking and the Role of ER Molecular Chaperones*

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