Expression of mutant Ins2C96Y results in enhanced tubule formation causing enlargement of pre-Golgi intermediates of CHO cells

Histochemistry and Cell Biology

Volumn 128, Issue 2, 2007, Pages 161-173

  Fan, Jing Yu ^a   Roth, Jürgen ^a   Zuber, Christian ^a  

^a UNIVERSITY OF ZURICH   (Switzerland)

Author keywords

Endoplasmic reticulum; Morphometry; Pre Golgi intermediates; Proinsulin; Protein quality control

Indexed keywords

PROINSULIN; PROTEASOME;

AGGRESOME; ANIMAL CELL; ARTICLE; CHO CELL; CONTROLLED STUDY; ELECTRON MICROSCOPY; ENDOPLASMIC RETICULUM; GOLGI COMPLEX; IMMUNOFLUORESCENCE; IMMUNOGOLD LABELING; MORPHOMETRICS; NONHUMAN; PANCREAS ISLET BETA CELL; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN FOLDING; QUALITY CONTROL; STATISTICAL SIGNIFICANCE; THREE DIMENSIONAL IMAGING; WILD TYPE; ANIMAL; CRICETULUS; DRUG ANTAGONISM; GENETICS; HAMSTER; IMMUNOHISTOCHEMISTRY; METABOLISM; MISSENSE MUTATION; MOUSE; ULTRASTRUCTURE;

MUS;

ANIMALS; CHO CELLS; CRICETINAE; CRICETULUS; ENDOPLASMIC RETICULUM; GOLGI APPARATUS; IMMUNOHISTOCHEMISTRY; MICE; MUTATION, MISSENSE; PROINSULIN; PROTEASOME ENDOPEPTIDASE COMPLEX;

EID: 34547609912     PISSN: 09486143     EISSN: None     Source Type: Journal    
DOI: 10.1007/s00418-007-0304-8     Document Type: Article

References (68)

1. Alanen A, Pira U, Lassila O, Roth J, Franklin R (1985) Mott cells are plasma cells defective in immunoglobulin secretion. Eur J Immunol 15:235-242
2. Allen JR, Nguyen LX, Sargent KE, Lipson KL, Hackett A, Urano F (2004) High ER stress in beta-cells stimulates intracellular degradation of misfolded insulin. Biochem Biophys Res Commun 324:166-170
3. Aridor M, Hannan LA (2002) Traffic jams II: an update of diseases of intracellular transport. Traffic 3:781-790
4. Bannykh SI, Balch WE (1997) Membrane dynamics at the endoplasmic reticulum-Golgi interface. J Cell Biol 138:1-4
5. Barral JM, Broadley SA, Schaffar G, Hartl FU (2004) Roles of molecular chaperones in protein misfolding diseases. Semin Cell Dev Biol 15:17-29
6. Caldwell SR, Hill KJ, Cooper AA (2001) Degradation of endoplasmic reticulum (ER) quality control substrates requires transport between the ER and Golgi. J Biol Chem 276:23296-23303
7. Carvalho P, Goder V, Rapoport TA (2006) Distinct ubiquitin-ligase complexes define convergent pathways for the degradation of ER proteins. Cell 126:361-373
8. Denic V, Quan EM, Weissman JS (2006) A luminal surveillance complex that selects misfolded glycoproteins for ER-associated degradation. Cell 126:349-359
9. Ellgaard L, Helenius A (2003) Quality control in the endoplasmic reticulum. Nat Rev Mol Cell Biol 4:181-191
10. Fabunmi RP, Wigley WC, Thomas PJ, DeMartino GN (2000) Activity and regulation of the centrosome-associated proteasome. J Biol Chem 275:409-413
11. Fan JY, Roth J, Zuber C (2003) Ultrastructural analysis of transitional endoplasmic reticulum and pre-Golgi intermediates: a highway for cars and trucks. Histochem Cell Biol 120:455-463
12. Farquhar M, Hauri H-P (1997) Protein sorting and vesicular traffic in the Golgi apparatus. In: Berger E, Roth J (eds) The Golgi apparatus. Birkhäuser Verlag, Basel, Boston, Berlin, pp 63-129
13. Garcia-Mata R, Gao YS, Sztul E (2002) Hassles with taking out the garbage: aggravating aggresomes. Traffic 3:388-396
14. Gilbert A, Jadot M, Leontieva E, Wattiaux D-C-S, Wattiaux R (1998) Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells. Exp Cell Res 242:144-152
15. Griffiths G, Pepperkok R, Locker JK, Kreis TE (1995) Immunocytochemical localization of beta-COP to the ER-Golgi boundary and the TGN. J Cell Sci 108:2839-2856
16. Halban PA, Irminger JC (2003) Mutant proinsulin that cannot be converted is secreted efficiently from primary rat beta-cells via the regulated pathway. Mol Biol Cell 14:1195-1203
17. Harding HP, Ron D (2002) Endoplasmic reticulum stress and the development of diabetes: a review. Diabetes 51(Suppl 3):S455-S461
18. Hauri HP, Kappeler F, Andersson H, Appenzeller C (2000) ERGIC-53 and traffic in the secretory pathway. J Cell Sci 113:587-596
19. Hirano K, Zuber C, Roth J, Ziak M (2003) The proteasome is involved in the degradation of different aquaporin-2 mutants causing nephrogenic diabetes insipidus. Am J Pathol 163:111-120
20. Hsu VW, Yuan LC, Nuchtern JG, Lippincott-Schwartz J, Hammerling GJ, Klausner RD (1991) A recycling pathway between the endoplasmic reticulum and the Golgi apparatus for retention of unassembled MHC class I molecules. Nature 352:441-444
21. Ismail N, Ng DT (2006) Have you HRD? Understanding ERAD is DOAble! Cell 126:237-239
22. Johnston JA, Ward CL, Kopito RR (1998) Aggresomes: a cellular response to misfolded proteins. J Cell Biol 143:1883-1898
23. Kayo T, Koizumi A (1998) Mapping of murine diabetogenic gene Mody on chromosome 7 at D7Mit258 and its involvement in pancreatic islet and b cell development during the perinatal period. J Clin Invest 101:2112-2118
24. Kim PS, Arvan P (1998) Endocrinopathies in the family of endoplasmic reticulum (ER) storage diseases: disorders of protein trafficking and the role of ER molecular chaperones. Endocr Rev 19:173-202
25. Kopito RR, Sitia R (2000) Aggresomes and Russell bodies. Symptoms of cellular indigestion? EMBO Rep 1:225-231
26. Lucocq J, Baschong W (1986) Preparation of protein colloidal gold complexes in the presence of commonly used buffers. Eur J Cell Biol 42:332-337
27. Lukacs GL, Mohamed A, Kartner N, Chang XB, Riordan JR, Grinstein S (1994) Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP. Embo J 13:6076-6086
28. Madsen OD, Frank BH, Steiner DF (1984) Human proinsulin-specific antigenic determinants identified by monoclonal antibodies. Diabetes 33:1012-1016
29. Mattioli L, Anelli T, Fagioli C, Tacchetti C, Sitia R, Valetti C (2006) ER storage diseases: a role for ERGIC-53 in controlling the formation and shape of Russell bodies. J Cell Sci 119:2532-2541
30. McNaught KSP, Shashidharan P, Perl DP, Jenner P, Olanow CW (2002) Aggresome-related biogenesis of Lewy bodies. Eur J Neurosci 16:2136-2148
31. MedeirosNeto G, Kim PS, Yoo SE, Vono J, Targovnik HM, Camargo R, Hossain SA, Arvan P (1996) Congenital hypothyroid goiter with deficient thyroglobulin-identification of an endoplasmic reticulum storage disease with induction of molecular chaperones. J Clin Invest 98:2838-2844
32. Meusser B, Hirsch C, Jarosch E, Sommer T (2005) ERAD: the long road to destruction. Nat Cell Biol 7:766-772
33. Moolenaar CEC, Ouwendijk J, Wittpoth M, Wisselaar HA, Hauri HP, Ginsel LA, Naim HY, Fransen JAM (1997) A mutation in a highly conserved region in brush-border sucrase-isomaltase and lysosomal alpha-glucosidase results in Golgi retention. J Cell Sci 110:557-567
34. Naim H, Roth J, Sterch E, Lentze M, Milla P, Schmitz J, Hauri H (1988) Sucrase-isomaltase deficiency in humans. Different mutations disrupt intracellular transport, processing, and function of an intestinal brush border enzyme. J Clin Invest 82:667-679
35. Orci L, Perrelet A, Ravazzola M, Amherdt M, Rothman JE, Schekman R (1994) Coatomer-rich endoplasmic reticulum. Proc Natl Acad Sci USA 91:11924-11928
36. Oyadomari S, Araki E, Mori M (2002a) Endoplasmic reticulum stress-mediated apoptosis in pancreatic beta-cells. Apoptosis 7:335-345
37. Oyadomari S, Koizumi A, Takeda K, Gotoh T, Akira S, Araki E, Mori M (2002b) Targeted disruption of the Chop gene delays endoplasmic reticulum stress-mediated diabetes. J Clin Invest 109:525-532
38. Palade G (1975) Intracellular aspects of the process of protein biosynthesis. Science 189:347-358
39. Pepperkok R, Scheel J, Horstmann H, Hauri HP, Griffiths G, Kreis TE (1993) beta-COP Is essential for biosynthetic membrane transport from the endoplasmic reticulum to the Golgi complex Invivo. Cell 74:71-82
40. Petäjä-Repo U, Hogue M, Laperrière A, Walkers P, Bouvier M (2000) Export from the endoplasmic reticulum represents the limiting step in the maturation and cell surface expression of the human d opiod receptor. J Biol Chem 275:13727-13736
41. Raposo G, van S-HM, Leijendekker R, Geuze HJ, Ploegh HL (1995) Misfolded major histocompatibility complex class I molecules accumulate in an expanded ER-Golgi intermediate compartment. J Cell Biol 131:1403-1419
42. Roth J, Bendayan M, Orci L (1978) Ultrastructural localization of intracellular antigens by the use of protein A-gold complex. J Histochem Cytochem 26:1074-1081
43. Roth J, Ziak M, Guhl B (2000) Non-heating antigen retrieval techniques for light and electron microscopic immunolabeling. In: Taylor CR, Shi S-R (eds) Antigen retrieval technique: a revolutionary approach to routine immunohistochemistry. Eaton Publishing, Natick, MA, pp 275-285
44. Roth J (2002) Protein N-glycosylation along the secretory pathway: relationship to organelle topography and function, protein quality control, and cell interactions. Chem Rev 102:285-303
45. Saraste J, Svensson K (1991) Distribution of intermediate elements operating in ER to Golgi transport. J Cell Sci 100:415-430
46. Schweizer A, Fransen J, Bächi T, Ginsel L, Hauri H (1988) Identification, by a monoclonal antibody, of a 53 kD protein associated with tubulovescular compartment at the cis-side of the Golgi apparatus. J Cell Biol 107:1643-1653
47. Schweizer A, Fransen JA, Matter K, Kreis TE, Ginsel L, Hauri HP (1990) Identification of an intermediate compartment involved in protein transport from endoplasmic reticulum to Golgi apparatus. Eur J Cell Biol 53:185-196
48. Sitia R, Braakman I (2003) Quality control in the endoplasmic reticulum protein factory. Nature 426:891-894
49. Tamarappoo BK, Verkman AS (1998) Defective aquaporin-2 trafficking in nephrogenic diabetes insipidus and correction by chemical chaperones. J Clin Invest 101:2257-2267
50. Tokuyasu K (1978) A study of positive staining of ultrathin frozen sections. J Ultrastruct Res 63:287-307
51. Tokuyasu K (1980a) Immunochemistry on ultrathin frozen sections. Histochem J 12:381-403
52. Tokuyasu K (1980b) Adsorption staining method for ultrathin frozen sections. In: Bailey G (ed) Proceedings of the 38th meeting of the electron microscopy society of America, Baton Rouge, pp 760-763
53. Torossi T, Fan JY, Sauter-Etter K, Roth J, Ziak M (2006) Endomannosidase processes oligosaccharides of alpha1-antitrypsin and its naturally occurring genetic variants in the Golgi apparatus. Cell Mol Life Sci 63:1923-1932
54. Valetti C, Grossi CE, Milstein C, Sitia R (1991) Russell bodies: a general response of secretory cells to synthesis of a mutant immunoglobulin which can neither exit from, nor be degraded in, the endoplasmic reticulum. J Cell Biol 115:983-994
55. Vanslyke JK, Deschenes SM, Musil LS (2000) Intracellular transport, assembly, and degradation of wild-type and disease-linked mutant gap junction proteins. Mol Biol Cell 11:1933-1946
56. Vashist S, Kim W, Belden WJ, Spear ED, Barlowe C, Ng DT (2001) Distinct retrieval and retention mechanisms are required for the quality control of endoplasmic reticulum protein folding. J Cell Biol 155:355-368
57. Vashist S, Ng DT (2004) Misfolded proteins are sorted by a sequential checkpoint mechanism of ER quality control. J Cell Biol 165:41-52
58. Wang J, Takeuchi T, Tanaka S, Kubo SK, Kayo T, Lu D, Takata K, Koizumi A, Izumi T (1999) A mutation in the insulin 2 gene induces diabetes with severe pancreatic beta-cell dysfunction in the Mody mouse. J Clin Invest 103:27-37
59. Ward CL, Kopito RR (1994) Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins. J Biol Chem 269:25710-25718
60. Weibel E (1979) Stereological methods. 1. Practical methods for biological morphometry: Academic, New York
61. Wigley WC, Fabunmi RP, Lee MG, Marino CR, Muallem S, DeMartino GN, Thomas PJ (1999) Dynamic association of proteasomal machinery with the centrosome. J Cell Biol 145:481-490
62. Wojcik C, Schroeter D, Wilk S, Lamprecht J, Paweletz N (1996) Ubiquitin-mediated proteolysis centers in HeLa cells: indication from studies of an inhibitor of the chymotrypsin-like activity of the proteasome. Eur J Cell Biol 71:311-318
63. Yam GH, Bosshard N, Zuber C, Steinmann B, Roth J (2006) Pharmacological chaperone corrects lysosomal storage in Fabry disease caused by trafficking-incompetent variants. Am J Physiol Cell Physiol 290:C1076-C1082
64. Yam GH, Gaplovska-Kysela K, Zuber C, Roth J (2007) Aggregated myocilin induces Russell bodies and causes apoptosis: implications for the pathogenesis of myocilin-caused primary open-angle glaucoma. Am J Pathol 170:100-109
65. Yoshioka M, Kayo T, Ikeda T, Koizumi A (1997) A novel locus, Mody4, distal to D7Mit189 on chromosome 7 determines early-onset NIDDM in nonobese C57BL/6J (Akita) mutant mice. Diabetes 46:887-894
66. Zatloukal K, Stumptner C, Fuchsbichler A, Heid H, Schnoelzer M, Kenner L, Kleinert R, Prinz M, Aguzzi A, Denk H (2002) p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases. Am J Pathol 160:255-263
67. Zuber C, Fan JY, Guhl B, Roth J (2004) Misfolded proinsulin accumulates in expanded pre-Golgi intermediates and endoplasmic reticulum subdomains in pancreatic beta cells of Akita mice. FASEB J 18:917-919
68. Zuber C, Cormier JH, Guhl B, Santimaria R, Hebert DN, Roth J (2007) EDEM1 reveals a quality control vesicular transport pathway out of the endoplasmic reticulum not involving the COPII exit sites. Proc Natl Acad Sci USA 104:4407-4412