HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Moussalli, M. Articles by Kaufman, R. J. Search for Related Content PubMed PubMed Citation Articles by Moussalli, M. Articles by Kaufman, R. J.

J Biol Chem, Vol. 274, Issue 46, 32539-32542, November 12, 1999

COMMUNICATION
Mannose-dependent Endoplasmic Reticulum (ER)-Golgi Intermediate Compartment-53-mediated ER to Golgi Trafficking of Coagulation Factors V and VIII

Micheline Moussalli, Steven W. Pipe^¶, Hans-Peter Hauri, William C. Nichols^**, David Ginsburg^§§, and Randal J. Kaufman^¶¶

From the Departments of ^¶ Pediatrics,  Medicine, ^§§ Human Genetics, and ^¶¶ Biological Chemistry, the  Howard Hughes Medical Institute, University of Michigan School of Medicine, Ann Arbor, Michigan 48109, the ^** Division of Human Genetics, Children's Hospital Medical Center, Cincinnatti, Ohio, and the  Department of Pharmacology/Neurobiology, Biozentrum, University of Basel, Basel CH-4056, Switzerland

The endoplasmic reticulum-Golgi intermediate compartment (ERGIC) is the site of segregation of secretory proteins for anterograde transport, via packaging into COPII-coated transport vesicles. ERGIC-53 is a homo-hexameric transmembrane lectin localized to the ERGIC that exhibits mannose-selective properties in vitro. Null mutations in ERGIC-53 were recently shown to be responsible for the autosomal recessive bleeding disorder, combined deficiency of coagulation factors V and VIII. We have studied the effect of defective ER to Golgi cycling by ERGIC-53 on the secretion of factors V and VIII. The secretion efficiency of factor V and factor VIII was studied in a tetracycline-inducible HeLa cell line overexpressing a wild-type ERGIC-53 or a cytosolic tail mutant of ERGIC-53 (KKAA) that is unable to exit the ER due to mutation of two COOH-terminal phenylalanine residues to alanines. The results show that efficient trafficking of factors V and VIII requires a functional ERGIC-53 cycling pathway and that this trafficking is dependent on post-translational modification of a specific cluster of asparagine (N)-linked oligosaccharides to a fully glucose-trimmed, mannose9 structure.

---

Copyright © 1999 by The American Society for Biochemistry and Molecular Biology, Inc.

This article has been cited by other articles:

				D. Yamaguchi, N. Kawasaki, I. Matsuo, K. Totani, H. Tozawa, N. Matsumoto, Y. Ito, and K. Yamamoto VIPL has sugar-binding activity specific for high-mannose-type N-glycans, and glucosylation of the {alpha}1,2 mannotriosyl branch blocks its binding Glycobiology, October 1, 2007; 17(10): 1061 - 1069. [Abstract] [Full Text] [PDF]

				T. Satoh, N. P. Cowieson, W. Hakamata, H. Ideo, K. Fukushima, M. Kurihara, R. Kato, K. Yamashita, and S. Wakatsuki Structural Basis for Recognition of High Mannose Type Glycoproteins by Mammalian Transport Lectin VIP36 J. Biol. Chem., September 21, 2007; 282(38): 28246 - 28255. [Abstract] [Full Text] [PDF]

				M. Renna, M. G. Caporaso, S. Bonatti, R. J. Kaufman, and P. Remondelli Regulation of ERGIC-53 Gene Transcription in Response to Endoplasmic Reticulum Stress J. Biol. Chem., August 3, 2007; 282(31): 22499 - 22512. [Abstract] [Full Text] [PDF]

				L. M. Ricketts, M. Dlugosz, K. B. Luther, R. S. Haltiwanger, and E. M. Majerus O-Fucosylation Is Required for ADAMTS13 Secretion J. Biol. Chem., June 8, 2007; 282(23): 17014 - 17023. [Abstract] [Full Text] [PDF]

				T. Satoh, K. Sato, A. Kanoh, K. Yamashita, Y. Yamada, N. Igarashi, R. Kato, A. Nakano, and S. Wakatsuki Structures of the Carbohydrate Recognition Domain of Ca2+-independent Cargo Receptors Emp46p and Emp47p J. Biol. Chem., April 14, 2006; 281(15): 10410 - 10419. [Abstract] [Full Text] [PDF]

				B. Zhang, B. McGee, J. S. Yamaoka, H. Guglielmone, K. A. Downes, S. Minoldo, G. Jarchum, F. Peyvandi, N. B. de Bosch, A. Ruiz-Saez, et al. Combined deficiency of factor V and factor VIII is due to mutations in either LMAN1 or MCFD2 Blood, March 1, 2006; 107(5): 1903 - 1907. [Abstract] [Full Text] [PDF]

				Y. Kamiya, Y. Yamaguchi, N. Takahashi, Y. Arata, K.-i. Kasai, Y. Ihara, I. Matsuo, Y. Ito, K. Yamamoto, and K. Kato Sugar-binding Properties of VIP36, an Intracellular Animal Lectin Operating as a Cargo Receptor J. Biol. Chem., November 4, 2005; 280(44): 37178 - 37182. [Abstract] [Full Text] [PDF]

				B. Zhang, R. J. Kaufman, and D. Ginsburg LMAN1 and MCFD2 Form a Cargo Receptor Complex and Interact with Coagulation Factor VIII in the Early Secretory Pathway J. Biol. Chem., July 8, 2005; 280(27): 25881 - 25886. [Abstract] [Full Text] [PDF]

				B. Nyfeler, S. W. Michnick, and H.-P. Hauri Capturing protein interactions in the secretory pathway of living cells PNAS, May 3, 2005; 102(18): 6350 - 6355. [Abstract] [Full Text] [PDF]

				C. Spatuzza, M. Renna, R. Faraonio, G. Cardinali, G. Martire, S. Bonatti, and P. Remondelli Heat Shock Induces Preferential Translation of ERGIC-53 and Affects Its Recycling Pathway J. Biol. Chem., October 8, 2004; 279(41): 42535 - 42544. [Abstract] [Full Text] [PDF]

				S. Svedine, T. Wang, R. Halaban, and D. N. Hebert Carbohydrates act as sorting determinants in ER-associated degradation of tyrosinase J. Cell Sci., June 15, 2004; 117(14): 2937 - 2949. [Abstract] [Full Text] [PDF]

				H. Z. Miao, N. Sirachainan, L. Palmer, P. Kucab, M. A. Cunningham, R. J. Kaufman, and S. W. Pipe Bioengineering of coagulation factor VIII for improved secretion Blood, May 1, 2004; 103(9): 3412 - 3419. [Abstract] [Full Text] [PDF]

				H. V. Yarovoi, D. Kufrin, D. E. Eslin, M. A. Thornton, S. L. Haberichter, Q. Shi, H. Zhu, R. Camire, S. S. Fakharzadeh, M. A. Kowalska, et al. Factor VIII ectopically expressed in platelets: efficacy in hemophilia A treatment Blood, December 1, 2003; 102(12): 4006 - 4013. [Abstract] [Full Text] [PDF]

				L. M. Velloso, K. Svensson, G. Schneider, R. F. Pettersson, and Y. Lindqvist Crystal Structure of the Carbohydrate Recognition Domain of p58/ERGIC-53, a Protein Involved in Glycoprotein Export from the Endoplasmic Reticulum J. Biol. Chem., May 3, 2002; 277(18): 15979 - 15984. [Abstract] [Full Text] [PDF]

				M. A. Skinner and A. G. Wildeman Suppression of Tumor-related Glycosylation of Cell Surface Receptors by the 16-kDa Membrane Subunit of Vacuolar H+-ATPase J. Biol. Chem., December 14, 2001; 276(51): 48451 - 48457. [Abstract] [Full Text] [PDF]

				M. Ermonval, C. Kitzmuller, A. M. Mir, R. Cacan, and N. E. Ivessa N-glycan structure of a short-lived variant of ribophorin I expressed in the MadIA214 glycosylation-defective cell line reveals the role of a mannosidase that is not ER mannosidase I in the process of glycoprotein degradation Glycobiology, July 1, 2001; 11(7): 565 - 576. [Abstract] [Full Text] [PDF]

				T. Dahm, J. White, S. Grill, J. Füllekrug, and E. H.K. Stelzer Quantitative ER {left-right-arrow} Golgi Transport Kinetics and Protein Separation upon Golgi Exit Revealed by Vesicular Integral Membrane Protein 36 Dynamics in Live Cells Mol. Biol. Cell, May 1, 2001; 12(5): 1481 - 1498. [Abstract] [Full Text]

				N. Y. Marcus and D. H. Perlmutter Glucosidase and Mannosidase Inhibitors Mediate Increased Secretion of Mutant alpha 1 Antitrypsin Z J. Biol. Chem., January 21, 2000; 275(3): 1987 - 1992. [Abstract] [Full Text] [PDF]

				H. Hauri, F Kappeler, H Andersson, and C Appenzeller ERGIC-53 and traffic in the secretory pathway J. Cell Sci., January 2, 2000; 113(4): 587 - 596. [Abstract] [PDF]

				M. A. Lehrman Oligosaccharide-based Information in Endoplasmic Reticulum Quality Control and Other Biological Systems J. Biol. Chem., March 16, 2001; 276(12): 8623 - 8626. [Full Text] [PDF]