Emergent properties of proteostasis-COPII coupled systems in human health and disease

Molecular Membrane Biology

Volumn 27, Issue 8, 2010, Pages 385-397

  Routledge, Katy E ^a   Gupta, Vijay ^a   Balch, William E ^a  

^a SCRIPPS RESEARCH INSTITUTE   (United States)

Author keywords

chaperone folding; COPII; inherited disease; membrane trafficking; proteostasis network

Indexed keywords

COAT PROTEIN COMPLEX II; GUANINE NUCLEOTIDE EXCHANGE FACTOR; GUANOSINE TRIPHOSPHATASE;

CELL BUDDING; CELL TYPE; ENDOPLASMIC RETICULUM; HUMAN; MEMBRANE TRANSPORT; PHENOTYPE; PRIORITY JOURNAL; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN HOMEOSTASIS; PROTEIN SYNTHESIS; REVIEW;

ANIMALS; COP-COATED VESICLES; GENETIC DISEASES, INBORN; HEALTH; HOMEOSTASIS; HUMANS; PROTEIN TRANSPORT; PROTEINS;

EUKARYOTA;

EID: 78650141466     PISSN: 09687688     EISSN: 14645203     Source Type: Journal    
DOI: 10.3109/09687688.2010.524894     Document Type: Review

References (103)

1. Annesi G, Aguglia U, Tarantino P, Annesi F, De Marco EV, Civitelli D, Torroni A, Quattrone A. 2007. SIL1 and SARA2 mutations in Marinesco-Sjogren and chylomicron retention diseases. Clin Genet 71:288-289.
2. Antonny B, Madden D, Hamamoto S, Orci L, Schekman R. 2001. Dynamics of the COPII coat with GTP and stable analogues. Nat Cell Biol 3:531-537.
3. Aridor M, Bannykh SI, Rowe T, Balch WE. 1999. Cargo can modulate COPII vesicle formation from the endoplasmic retic-ulum. J Biol Chem 274:4389-4399.
4. Aridor M, Fish KN, Bannykh S, Weissman J, Roberts TH, Lippincott-Schwartz J, Balch WE. 2001. The Sar1 GTPase coordinates biosynthetic cargo selection with endoplasmic retic-ulum export site assembly. J Cell Biol 152:213-229.
5. Aridor M, Weissman J, Bannykh S, Nuoffer C, Balch WE. 1998. Cargo selection by the COPII budding machinery during export from the ER. J Cell Biol 141:61-70.
6. Balch WE, Morimoto RI, Dillin A, Kelly JW. 2008. Adapting proteostasis for disease intervention. Science 319: 916-919.
7. Bentley M, Nycz DC, Joglekar A, Fertschai I, Malli R, Graier WF, Hay JC. 2010. Vesicular calcium regulates coat retention, fuso-genicity, and size of pre-Golgi intermediates. Mol Biol Cell 21:1033-1046.
8. Bi X, Corpina RA, Goldberg J. 2002. Structure of the Sec23/24- Sar1 pre-budding complex of the COPII vesicle coat. Nature 419:271-277.
9. Bi X, Mancias JD, Goldberg J. 2007. Insights into COPII coat nucleation from the structure of Sec23.Sar1 complexed with the active fragment of Sec31. Dev Cell 13:635-645.
10. Bielli A, Haney CJ, Gabreski G, Watkins SC, Bannykh SI, Aridor M. 2005. Regulation of Sar1 NH2 terminus by GTP binding and hydrolysis promotes membrane deformation to control COPII vesicle fission. J Cell Biol 171:919-924.
11. Blumental-Perry A, Haney CJ, Weixel KM, Watkins SC, Weisz OA, Aridor M. 2006. Phosphatidylinositol 4-phosphate formation at ER exit sites regulates ER export. Dev Cell 11: 671-682.
12. Bonnon C, Wendeler MW, Paccaud JP, Hauri HP. 2010. Selective export of human GPI-anchored proteins from the endoplasmic reticulum. J Cell Sci 123:1705-1715.
13. Boyadjiev SA, Fromme JC, Ben J, Chong SS, Nauta C, Hur DJ, Zhang G, Hamamoto S, Schekman R, Ravazzola M, et al. 2006. Cranio-lenticulo-sutural dysplasia is caused by a SEC23A mutation leading to abnormal endoplasmic-reticulum-to-Golgi trafficking. Nat Genet 38:1192-1197.
14. Brewer JW, Hendershot LM. 2005. Building an antibody factory: A job for the unfolded protein response. Nat Immunol 6:23-29.
15. Brown WJ, Plutner H, Drecktrah D, Judson BL, Balch WE. 2008. The lysophospholipid acyltransferase antagonist CI-976 inhibits a late step in COPII vesicle budding. Traffic 9:786-797.
16. Budnik A, Stephens DJ. 2009. ER exit sites - localization and control of COPII vesicle formation. FEBS Lett 583:3796-3803.
17. Cai H, Yu S, Menon S, Cai Y, Lazarova D, Fu C, Reinisch K, Hay JC, Ferro-Novick S. 2007. TRAPPI tethers COPII vesicles by binding the coat subunit Sec23. Nature 445: 941-944.
18. Charcosset M, Sassolas A, Peretti N, Roy CC, Deslandres C, Sinnett D, Levy E, Lachaux A. 2008. Anderson or chylomicron retention disease: Molecular impact of five mutations in the SAR1B gene on the structure and the functionality of Sar1b protein. Mol Genet Metab 93:74-84.
19. Connerly PL, Esaki M, Montegna EA, Strongin DE, Levi S, Soderholm J, Glick BS. 2005. Sec16 is a determinant of transitional ER organization. Curr Biol 15:1439-1447.
20. Cuervo AM. 2008. Autophagy and aging: Keeping that old broom working. Trends Genet 24:604-612.
21. Dancourt J, Barlowe C. 2010. Protein sorting receptors in the early secretory pathway. Annu Rev Biochem 79:777-802.
22. Espenshade P, Gimeno RE, Holzmacher E, Teung P, Kaiser CA. 1995. Yeast SEC16 gene encodes a multidomain vesicle coat protein that interacts with Sec23p. J Cell Biol 131:311-324.
23. Farhan H, Weiss M, Tani K, Kaufman RJ, Hauri HP. 2008. Adaptation of endoplasmic reticulum exit sites to acute and chronic increases in cargo load. EMBO J 27:2043-2054.
24. Farhan H, Wendeler MW, Mitrovic S, Fava E, Silberberg Y, Sharan R, Zerial M, Hauri HP. 2010. MAPK signaling to the early secretory pathway revealed by kinase/phosphatase functional screening. J Cell Biol 189:997-1011.
25. Faso C, Chen YN, Tamura K, Held M, Zemelis S, Marti L, Saravanan R, Hummel E, Kung L, Miller E, et al. 2009. A missense mutation in the Arabidopsis COPII coat protein sec24a induces the formation of clusters of the endoplasmic reticulum and golgi apparatus. Plant Cell 21:3655-3671.
26. Fath S, Mancias JD, Bi X, Goldberg J. 2007. Structure and organization of coat proteins in the COPII cage. Cell 129:1325-1336.
27. Finley D. 2009. Recognition and processing of ubiquitin-protein conjugates by the proteasome. Annu Rev Biochem 78: 477-513.
28. Forster R, Weiss M, Zimmermann T, Reynaud EG, Verissimo F, Stephens DJ, Pepperkok R. 2006. Secretory cargo regulates the turnover of COPII subunits at single ER exit sites. Curr Biol 16:173-179.
29. Fromme JC, Orci L, Schekman R. 2008. Coordination of COPII vesicle trafficking by Sec23. Trends Cell Biol 18:330-336.
30. Fromme JC, Ravazzola M, Hamamoto S, Al-Balwi M, Eyaid W, Boyadjiev SA, Cosson P, Schekman R, Orci L. 2007. The genetic basis of a craniofacial disease provides insight into COPII coat assembly. Dev Cell 13:623-634.
31. Fromme JC, Schekman R. 2005. COPII-coated vesicles: Flexible enough for large cargo Curr Opin Cell Biol 17:345-352.
32. Gidalevitz T, Kikis EA, Morimoto RI. 2010. A cellular perspective on conformational disease: The role of genetic background and proteostasis networks. Curr Opin Struct Biol 20:23-32.
33. Guo Y, Linstedt AD. 2006. COPII-Golgi protein interactions regulate COPII coat assembly and Golgi size. J Cell Biol 174:53-63.
34. Gupta V, Palmer KJ, Spence P, Hudson A, Stephens DJ. 2008. Kinesin-1 (uKHC/KIF5B) is required for bidirectional motility of ER exit sites and efficient ER-to-Golgi transport. Traffic 9:1850-1866.
35. Gurkan C, Koulov AV, Balch WE. 2007. An evolutionary perspective on eukaryotic membrane trafficking. Adv Exp Med Biol 607:73-83.
36. Gurkan C, Stagg SM, Lapointe P, Balch WE. 2006. The COPII cage: Unifying principles of vesicle coat assembly. Nat Rev Mol Cell Biol 7:727-738.
37. Heidtman M, Chen CZ, Collins RN, Barlowe C. 2003. A role for Yip1p in COPII vesicle biogenesis. J Cell Biol 163:57-69.
38. Heinzer S, Worz S, Kalla C, Rohr K, Weiss M. 2008. A model for the self-organization of exit sites in the endoplasmic reticulum. J Cell Sci 121:55-64.
39. Higashio H, Sato K, Nakano A. 2008. Smy2p participates in COPII vesicle formation through the interaction with Sec23p/Sec24p subcomplex. Traffic 9:79-93.
40. Hughes H, Budnik A, Schmidt K, Palmer KJ, Mantell J, Noakes C, Johnson A, Carter DA, Verkade P, Watson P, et al. 2009. Organisation of human ER-exit sites: Requirements for the localisation of Sec16 to transitional ER. J Cell Sci 122: 2924-2934.
41. Hutt D, Balch WE. 2010. Cell biology. The proteome in balance. Science 329:766-767.
42. Hutt DM, Powers ET, Balch WE. 2009. The proteostasis boundary in misfolding diseases of membrane traffic. FEBS Lett 583:2639-2646.
43. Iolascon A, D'Agostaro G, Perrotta S, Izzo P, Tavano R, Miraglia del Giudice B. 1996. Congenital dyserythropoietic anemia type II: Molecular basis and clinical aspects. Haematologica 81:543-559.
44. Iolascon A, Russo R, Esposito MR, Asci R, Piscopo C, Perrotta S, Feneant-Thibault M, Garcon L, Delaunay J. 2009. Molecular analysis of forty two Cda Ii patients: New mutations in the Sec23b gene Search for a genotype-phenotype relationship. Haematologica 95:708-715.
45. Jo Y, Debose-Boyd RA. 2010. Control of cholesterol synthesis through regulated ER-associated degradation of HMG CoA reductase. Crit Rev Biochem Mol Biol 45:185-198.
46. Jones B, Jones EL, Bonney SA, Patel HN, Mensenkamp AR, Eichenbaum-Voline S, Rudling M, Myrdal U, Annesi G, Naik S, et al. 2003. Mutations in a Sar1 GTPase of COPII vesicles are associated with lipid absorption disorders. Nat Genet 34:29-31.
47. Kapetanovich L, Baughman C, Lee TH. 2005. Nm23H2 facilitates coat protein complex II assembly and endoplasmic reticulum export in mammalian cells. Mol Biol Cell 16:835-848.
48. Konstantinova IM, Tsimokha AS, Mittenberg AG. 2008. Role of proteasomes in cellular regulation. Int Rev Cell Mol Biol 267:59-124.
49. Korolchuk VI, Menzies FM, Rubinsztein DC. 2010. Mechanisms of cross-talk between the ubiquitin-proteasome and autopha-gy-lysosome systems. FEBS Lett 584:1393-1398.
50. Lang MR, Lapierre LA, Frotscher M, Goldenring JR, Knapik EW. 2006. Secretory COPII coat component Sec23a is essential for craniofacial chondrocyte maturation. Nat Genet 38:1198-1203.
51. Lee C, Goldberg J. 2010. Structure of coatomer cage proteins and the relationship among COPI, COPII, and clathrin vesicle coats. Cell 142:123-132.
52. Lee MC, Orci L, Hamamoto S, Futai E, Ravazzola M, Schekman R. 2005. Sar1p N-terminal helix initiates membrane curvature and completes the fission of a COPII vesicle. Cell 122:605-617.
53. Long KR, Yamamoto Y, Baker AL, Watkins SC, Coyne CB, Conway JF, Aridor M. 2010. Sar1 assembly regulates membrane constriction and ER export. J Cell Biol 190: 115-128.
54. Mancias JD, Goldberg J. 2008. Structural basis of cargo membrane protein discrimination by the human COPII coat machinery. EMBO J 27:2918-2928.
55. Matsuoka K, Orci L, Amherdt M, Bednarek SY, Hamamoto S, Schekman R, Yeung T. 1998. COPII-coated vesicle formation reconstituted with purified coat proteins and chemically defined liposomes. Cell 93:263-275.
56. Merte J, Jensen D, Wright K, Sarsfield S, Wang Y, Schekman R, Ginty DD. 2010. Sec24b selectively sorts Vangl2 to regulate planar cell polarity during neural tube closure. Nat Cell Biol 12:41-46.
57. Miller EA, Beilharz TH, Malkus PN, Lee MC, Hamamoto S, Orci L, Schekman R. 2003. Multiple cargo binding sites on the COPII subunit Sec24p ensure capture of diverse membrane proteins into transport vesicles. Cell 114:497-509.
58. Mori K. 2009. Signalling pathways in the unfolded protein response: Development from yeast to mammals. J Biochem 146:743-750.
59. Morimoto RI. 2008. Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging. Genes Dev 22:1427-1438.
60. Mossessova E, Bickford LC, Goldberg J. 2003. SNARE selectivity of the COPII coat. Cell 114:483-495.
61. Mu TW, Ong DS, Wang YJ, Balch WE, Yates JR 3rd, Segatori L, Kelly JW. 2008. Chemical and biological approaches synergize to ameliorate protein-folding diseases. Cell 134:769-781.
62. Ohisa S, Inohaya K, Takano Y, Kudo A. 2010. Sec24d encoding a component of COPII is essential for vertebra formation, revealed by the analysis of the medaka mutant, vbi. Dev Biol 342:85-95.
63. Ong YS, Tang BL, Loo LS, Hong W. 2010. P125A exists as part of the mammalian Sec13/Sec31 COPII subcomplex to facilitate ER-Golgi transport. J Cell Biol 190:331-345.
64. Palmer KJ, Konkel JE, Stephens DJ. 2005. PCTAIRE protein kinases interact directly with the COPII complex and modulate secretory cargo transport. J Cell Sci 118:3839-3847.
65. Panowski SH, Dillin A. 2009. Signals of youth: Endocrine regulation of aging in Caenorhabditis elegans. Trends Endocrinol Metab 20:259-264.
66. Pathre P, Shome K, Blumental-Perry A, Bielli A, Haney CJ, Alber S, Watkins SC, Romero G, Aridor M. 2003. Activation of phospholipase D by the small GTPase Sar1p is required to support COPII assembly and ER export. EMBO J 22: 4059-4069.
67. Petersen OH, Michalak M, Verkhratsky A. 2005. Calcium signalling: Past, present and future. Cell Calcium 38:161-169.
68. Powers ET, Morimoto RI, Dillin A, Kelly JW, Balch WE. 2009. Biological and chemical approaches to diseases of proteostasis deficiency. Annu Rev Biochem 78:959-991.
69. Prahlad V, Morimoto RI. 2009. Integrating the stress response: Lessons for neurodegenerative diseases from C. elegans. Trends Cell Biol 19:52-61.
70. Rismanchi N, Puertollano R, Blackstone C. 2009. STAM adaptor proteins interact with COPII complexes and function in ER-to-Golgi trafficking. Traffic 10:201-217.
71. Ron D, Walter P. 2007. Signal integration in the endoplasmic reticulum unfolded protein response. Nat Rev Mol Cell Biol 8:519-529.
72. Rossanese OW, Soderholm J, Bevis BJ, Sears IB, O'Connor J, Williamson EK, Glick BS. 1999. Golgi structure correlates with transitional endoplasmic reticulum organization in Pichia pas-toris and Saccharomyces cerevisiae. J Cell Biol 145:69-81.
73. Roy L, Bergeron JJ, Lavoie C, Hendriks R, Gushue J, Fazel A, Pelletier A, Morre DJ, Subramaniam VN, Hong W, et al. 2000. Role of p97 and syntaxin 5 in the assembly of transitional endoplasmic reticulum. Mol Biol Cell 11:2529-2542.
74. Russell C, Stagg SM. 2010. New insights into the structural mechanisms of the COPII coat. Traffic 11:303-310.
75. Saito K, Chen M, Bard F, Chen S, Zhou H, Woodley D, Polischuk R, Schekman R, Malhotra V. 2009. TANGO1 facilitates cargo loading at endoplasmic reticulum exit sites. Cell 136:891-902.
76. Saito-Nakano Y, Nakano A. 2000. Sed4p functions as a positive regulator of Sar1p probably through inhibition of the GTPase activation by Sec23p. Genes Cells 5:1039-1048.
77. Schwarz K, Iolascon A, Verissimo F, Trede NS, Horsley W, Chen W, Paw BH, Hopfner KP, Holzmann K, Russo R, et al. 2009. Mutations affecting the secretory COPII coat component SEC23B cause congenital dyserythropoietic anemia type II. Nat Genet 41:936-940.
78. Sekijima Y, Wiseman RL, Matteson J, Hammarstrom P, Miller SR, Sawkar AR, Balch WE, Kelly JW. 2005. The biological and chemical basis for tissue-selective amyloid disease. Cell 121: 73-85.
79. Settles EI, Loftus AF, Mckeown AN, Parthasarathy R. 2010. The vesicle trafficking protein Sar1 lowers lipid membrane rigidity. Biophys J 99:1539-1545.
80. Shamovsky I, Nudler E. 2008. New insights into the mechanism of heat shock response activation. Cell Mol Life Sci 65: 855-861.
81. Shimoi W, Ezawa I, Nakamoto K, Uesaki S, Gabreski G, Aridor M, Yamamoto A, Nagahama M, Tagaya M, Tani K. 2005. P125 is localized in endoplasmic reticulum exit sites and involved in their organization. J Biol Chem 280:10141-10148.
82. Siddiqi S, Siddiqi SA, Mansbach CM 2nd. 2009. SEC24C is required for the docking of the pre-chylomicron transport vesicle with the Golgi. J Lipid Res 51:1093-1100.
83. Stagg SM, Gurkan C, Fowler DM, LaPointe P, Foss TR, Potter CS, Carragher B, Balch WE. 2006. Structure of the Sec13/31 COPII coat cage. Nature 439:234-238.
84. Stagg SM, LaPointe P, Balch WE. 2007. Structural design of cage and coat scaffolds that direct membrane traffic. Curr Opin Struct Biol 17:221-228.
85. Stagg SM, LaPointe P, Razvi A, Gurkan C, Potter CS, Carragher B, Balch WE. 2008. Structural basis for cargo regulation of COPII coat assembly. Cell 134:474-484.
86. Sun LP, Seemann J, Goldstein JL, Brown MS. 2007. Sterol-regulated transport of SREBPs from endoplasmic reticulum to Golgi: Insig renders sorting signal in Scap inaccessible to COPII proteins. Proc Natl Acad Sci USA 104:6519-6526.
87. Tabata KV, Sato K, Ide T, Nishizaka T, Nakano A, Noji H. 2009. Visualization of cargo concentration by COPII minimal machinery in a planar lipid membrane. EMBO J 28:3279-3289.
88. Tang BL, Ong YS, Huang B, Wei S, Wong ET, Qi R, Horstmann H, Hong W. 2001. A membrane protein enriched in endoplasmic reticulum exit sites interacts with COPII. J Biol Chem 276:40008-40017.
89. Tani K, Mizoguchi T, Iwamatsu A, Hatsuzawa K, Tagaya M. 1999. p125 is a novel mammalian Sec23p-interacting protein with structural similarity to phospholipid-modifying proteins. J Biol Chem 274:20505-20512.
90. Townley AK, Feng Y, Schmidt K, Carter DA, Porter R, Verkade P, Stephens DJ. 2008. Efficient coupling of Sec23-Sec24 to Sec13-Sec31 drives COPII-dependent collagen secretion and is essential for normal craniofacial development. J Cell Sci 121:3025-3034.
91. Trahey M, Hay JC. 2010. Transport vesicle uncoating: It's later than you think. F1000 Biol Rep 2:47.
92. Treepongkaruna S, Chongviriyaphan N, Suthutvoravut U, Charoenpipop D, Choubtum L, Wattanasirichaigoon D. 2009. Novel missense mutations of SAR1B gene in an infant with chylomicron retention disease. J Pediatr Gastroenterol Nutr 48:370-373.
93. Wang L, Lucocq JM. 2007. p38 MAPK regulates COPII recruitment. Biochem Biophys Res Commun 363:317-321.
94. Wang X, Matteson J, An Y, Moyer B, Yoo JS, Bannykh S, Wilson IA, Riordan JR, Balch WE. 2004. COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code. J Cell Biol 167:65-74.
95. Watson P, Forster R, Palmer KJ, Pepperkok R, Stephens DJ. 2005. Coupling of ER exit to microtubules through direct interaction of COPII with dynactin. Nat Cell Biol 7:48-55.
96. Watson P, Townley AK, Koka P, Palmer KJ, Stephens DJ. 2006. Sec16 defines endoplasmic reticulum exit sites and is required for secretory cargo export in mammalian cells. Traffic 7: 1678-1687.
97. Wendeler MW, Paccaud JP, Hauri HP. 2007. Role of Sec24 isoforms in selective export of membrane proteins from the endoplasmic reticulum. EMBO Rep 8:258-264.
98. Wiseman RL, Koulov A, Powers E, Kelly JW, Balch WE. 2007a. Protein energetics in maturation of the early secretory pathway. Curr Opin Cell Biol 19:359-367.
99. Wiseman RL, Powers ET, Buxbaum JN, Kelly JW, Balch WE. 2007b. An adaptable standard for protein export from the endoplasmic reticulum. Cell 131:809-821.
100. Yamasaki A, Tani K, Yamamoto A, Kitamura N, Komada M. 2006. The Ca2+-binding protein ALG-2 is recruited to endoplasmic reticulum exit sites by Sec31A and stabilizes the localization of Sec31A. Mol Biol Cell 17:4876-4887.
101. Yang Z, Klionsky DJ. 2010. Mammalian autophagy: Core molecular machinery and signaling regulation. Curr Opin Cell Biol 22:124-131.
102. Yellaturu CR, Deng X, Cagen LM, Wilcox HG, Mansbach CM 2nd, Siddiqi SA, Park EA, Raghow R, Elam MB. 2009. Insulin enhances post-translational processing of nascent SREBP-1c by promoting its phosphorylation and association with COPII vesicles. J Biol Chem 284: 7518-7532.
103. Zhang K, Kaufman RJ. 2008. From endoplasmic-reticulum stress to the inflammatory response. Nature 454:455-462.