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 Web of Science 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 Web of Science (54) Citing Articles via Google Scholar Google Scholar Articles by Hebert, D. N. Articles by Molinari, M. Search for Related Content PubMed PubMed Citation Articles by Hebert, D. N. Articles by Molinari, M.

In and Out of the ER: Protein Folding, Quality Control, Degradation, and Related Human Diseases

Institute for Research in Biomedicine, Bellinzona, Switzerland; and Department of Biochemistry and Molecular Biology, Program in Molecular and Cellular Biology, University of Massachusetts, Amherst, Massachusetts

A substantial fraction of eukaryotic gene products are synthesized by ribosomes attached at the cytosolic face of the endoplasmic reticulum (ER) membrane. These polypeptides enter cotranslationally in the ER lumen, which contains resident molecular chaperones and folding factors that assist their maturation. Native proteins are released from the ER lumen and are transported through the secretory pathway to their final intra- or extracellular destination. Folding-defective polypeptides are exported across the ER membrane into the cytosol and destroyed. Cellular and organismal homeostasis relies on a balanced activity of the ER folding, quality control, and degradation machineries as shown by the dozens of human diseases related to defective maturation or disposal of individual polypeptides generated in the ER.

This article has been cited by other articles:

				R. P Quinn, S. J Mahoney, B. M Wilkinson, D. J Thornton, and C. J Stirling A novel role for Gtb1p in glucose trimming of N-linked glycans Glycobiology, December 1, 2009; 19(12): 1408 - 1416. [Abstract] [Full Text] [PDF]

				P. Roboti, E. Swanton, and S. High Differences in endoplasmic-reticulum quality control determine the cellular response to disease-associated mutants of proteolipid protein J. Cell Sci., November 1, 2009; 122(21): 3942 - 3953. [Abstract] [Full Text] [PDF]

				P. J. Lim, R. Danner, J. Liang, H. Doong, C. Harman, D. Srinivasan, C. Rothenberg, H. Wang, Y. Ye, S. Fang, et al. Ubiquilin and p97/VCP bind erasin, forming a complex involved in ERAD J. Cell Biol., October 19, 2009; 187(2): 201 - 217. [Abstract] [Full Text] [PDF]

				S. D. Whitman, E. C. Smith, and R. E. Dutch Differential Rates of Protein Folding and Cellular Trafficking for the Hendra Virus F and G Proteins: Implications for F-G Complex Formation J. Virol., September 1, 2009; 83(17): 8998 - 9001. [Abstract] [Full Text] [PDF]

				J. Cui, T. Smith, P. W. Robbins, and J. Samuelson Darwinian selection for sites of Asn-linked glycosylation in phylogenetically disparate eukaryotes and viruses PNAS, August 11, 2009; 106(32): 13421 - 13426. [Abstract] [Full Text] [PDF]

				N. Zaarour, S. Demaretz, N. Defontaine, D. Mordasini, and K. Laghmani A Highly Conserved Motif at the COOH Terminus Dictates Endoplasmic Reticulum Exit and Cell Surface Expression of NKCC2 J. Biol. Chem., August 7, 2009; 284(32): 21752 - 21764. [Abstract] [Full Text] [PDF]

				E. White, J. McKenna, A. Cavanaugh, and G. E. Breitwieser Pharmacochaperone-Mediated Rescue of Calcium-Sensing Receptor Loss-of-Function Mutants Mol. Endocrinol., July 1, 2009; 23(7): 1115 - 1123. [Abstract] [Full Text] [PDF]

				M. H. Doolittle, O. Ben-Zeev, S. Bassilian, J. P. Whitelegge, M. Peterfy, and H. Wong Hepatic lipase maturation: a partial proteome of interacting factors J. Lipid Res., June 1, 2009; 50(6): 1173 - 1184. [Abstract] [Full Text] [PDF]

				P. B. Beauregard, R. Guerin, C. Turcotte, S. Lindquist, and L. A. Rokeach A nucleolar protein allows viability in the absence of the essential ER-residing molecular chaperone calnexin J. Cell Sci., May 1, 2009; 122(9): 1342 - 1351. [Abstract] [Full Text] [PDF]

				A. Mishra, S. K. Godavarthi, M. Maheshwari, A. Goswami, and N. R. Jana The Ubiquitin Ligase E6-AP Is Induced and Recruited to Aggresomes in Response to Proteasome Inhibition and May Be Involved in the Ubiquitination of Hsp70-bound Misfolded Proteins J. Biol. Chem., April 17, 2009; 284(16): 10537 - 10545. [Abstract] [Full Text] [PDF]

				D. J. Termine, K. W. Moremen, and R. N. Sifers The mammalian UPR boosts glycoprotein ERAD by suppressing the proteolytic downregulation of ER mannosidase I J. Cell Sci., April 1, 2009; 122(7): 976 - 984. [Abstract] [Full Text] [PDF]

				M. C. Jonikas, S. R. Collins, V. Denic, E. Oh, E. M. Quan, V. Schmid, J. Weibezahn, B. Schwappach, P. Walter, J. S. Weissman, et al. Comprehensive Characterization of Genes Required for Protein Folding in the Endoplasmic Reticulum Science, March 27, 2009; 323(5922): 1693 - 1697. [Abstract] [Full Text] [PDF]

				O. Vagin, J. A. Kraut, and G. Sachs Role of N-glycosylation in trafficking of apical membrane proteins in epithelia Am J Physiol Renal Physiol, March 1, 2009; 296(3): F459 - F469. [Abstract] [Full Text] [PDF]

				L. Zhang, E. Lai, T. Teodoro, and A. Volchuk GRP78, but Not Protein-disulfide Isomerase, Partially Reverses Hyperglycemia-induced Inhibition of Insulin Synthesis and Secretion in Pancreatic {beta}-Cells J. Biol. Chem., February 20, 2009; 284(8): 5289 - 5298. [Abstract] [Full Text] [PDF]

				L. Izquierdo, A. Atrih, J. A. Rodrigues, D. C. Jones, and M. A. J. Ferguson Trypanosoma brucei UDP-Glucose:Glycoprotein Glucosyltransferase Has Unusual Substrate Specificity and Protects the Parasite from Stress Eukaryot. Cell, February 1, 2009; 8(2): 230 - 240. [Abstract] [Full Text] [PDF]

				G. L. Vilas, D. E. Johnson, P. Freund, and J. R. Casey Characterization of an epilepsy-associated variant of the human Cl-/HCOFormula exchanger AE3 Am J Physiol Cell Physiol, January 1, 2009; 297(3): C526 - C536. [Abstract] [Full Text] [PDF]

				N. Wang, E. J. Glidden, S. R. Murphy, B. R. Pearse, and D. N. Hebert The Cotranslational Maturation Program for the Type II Membrane Glycoprotein Influenza Neuraminidase J. Biol. Chem., December 5, 2008; 283(49): 33826 - 33837. [Abstract] [Full Text] [PDF]

				M. H. Brush and S. Shenolikar Control of Cellular GADD34 Levels by the 26S Proteasome Mol. Cell. Biol., December 1, 2008; 28(23): 6989 - 7000. [Abstract] [Full Text] [PDF]

				C.-H. Chan, H. M. Mitchison, and D. A. Pearce Transcript and in silico analysis of CLN3 in juvenile neuronal ceroid lipofuscinosis and associated mouse models Hum. Mol. Genet., November 1, 2008; 17(21): 3332 - 3339. [Abstract] [Full Text] [PDF]

				O. Vanoni, P. Paganetti, and M. Molinari Consequences of Individual N-glycan Deletions and of Proteasomal Inhibition on Secretion of Active BACE Mol. Biol. Cell, October 1, 2008; 19(10): 4086 - 4098. [Abstract] [Full Text] [PDF]

				A. Ashok and R. S. Hegde Retrotranslocation of Prion Proteins from the Endoplasmic Reticulum by Preventing GPI Signal Transamidation Mol. Biol. Cell, August 1, 2008; 19(8): 3463 - 3476. [Abstract] [Full Text] [PDF]

				R. Bernasconi, T. Pertel, J. Luban, and M. Molinari A Dual Task for the Xbp1-responsive OS-9 Variants in the Mammalian Endoplasmic Reticulum: INHIBITING SECRETION OF MISFOLDED PROTEIN CONFORMERS AND ENHANCING THEIR DISPOSAL J. Biol. Chem., June 13, 2008; 283(24): 16446 - 16454. [Abstract] [Full Text] [PDF]

				B. R. Pearse, L. Gabriel, N. Wang, and D. N. Hebert A cell-based reglucosylation assay demonstrates the role of GT1 in the quality control of a maturing glycoprotein J. Cell Biol., April 21, 2008; 181(2): 309 - 320. [Abstract] [Full Text] [PDF]

				L. Abrami, B. Kunz, I. Iacovache, and F. G. van der Goot Palmitoylation and ubiquitination regulate exit of the Wnt signaling protein LRP6 from the endoplasmic reticulum PNAS, April 8, 2008; 105(14): 5384 - 5389. [Abstract] [Full Text] [PDF]