Malfolded protein structure and proteostasis in lung diseases

American Journal of Respiratory and Critical Care Medicine

Volumn 189, Issue 1, 2014, Pages 96-103

  Balch, William E ^a   Sznajder, Jacob I ^b   Budinger, Scott ^b   Finley, Daniel ^c   Laposky, Aaron D ^d   Cuervo, Ana Maria ^e   Benjamin, Ivor J ^f   Barreiro, Esther ^g   Morimoto, Richard I ^h   Postow, Lisa ^d   Weissman, Allan M ⁱ   Gail, Dorothy ^d   Banks Schlegel, Susan ^d   Croxton, Thomas ^d   Gan, Weiniu ^d  

^a SCRIPPS RESEARCH INSTITUTE   (United States)

^b NORTHWESTERN UNIVERSITY   (United States)

^c HARVARD MEDICAL SCHOOL   (United States)

^d NATIONAL HEART LUNG AND BLOOD INSTITUTE   (United States)

^e ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

^f MEDICAL COLLEGE OF WISCONSIN   (United States)

^g INST MUNIC D INVESTIGACIO MEDICA   (Spain)

^h NORTHWESTERN UNIVERSITY   (United States)

ⁱ NATIONAL CANCER INSTITUTE   (United States)

Author keywords

Post translational processing; Protein misfolding; Proteosome; Pulmonary health; Ubiquitination

Indexed keywords

AIRWAY; ARTICLE; HEALTH; HUMAN; LUNG DISEASE; MALFOLDED PROTEIN STRUCTURE; NONHUMAN; PATHOLOGY; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN FOLDING; PROTEIN HOMEOSTASIS; PROTEIN STRUCTURE; SCIENCE;

EID: 84891868645     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: None     Document Type: Article

References (65)

1. Budinger GR, Sznajder JI. The alveolar-epithelial barrier: a target for potential therapy [abstract ix]. Clin Chest Med 2006;27: 655-669.
2. Hoyert DL, Xu J. Deaths: preliminary data for 2011. Natl Vital Stat Rep 2012;61(6):1-52.
3. Centers for Disease Control and Prevention (CDC). Chronic obstructive pulmonary disease among adults-United States, 2011. MMWR Morb Mortal Wkly Rep 2012;61:938-943.
4. Bouchecareilh M, Balch WE. Proteostasis: a new therapeutic paradigm for pulmonary disease. Proc Am Thorac Soc 2011;8:189-195.
5. Balch WE, Morimoto RI, Dillin A, Kelly JW. Adapting proteostasis for disease intervention. Science 2008;319:916-919.
6. Kikis EA, Gidalevitz T, Morimoto RI. Protein homeostasis in models of aging and age-related conformational disease. Adv Exp Med Biol 2010;694:138-159.
7. Hutt DM, Balch WE. Expanding proteostasis by membrane trafficking networks. Cold Spring Harb Perspect Biol 2013;5.
8. Powers ET, Balch WE. Diversity in the origins of proteostasis networks-a driver for protein function in evolution. Nat Rev Mol Cell Biol 2013; 14:237-248.
9. Hebert DN, Molinari M. In and out of the ER: protein folding, quality control, degradation, and related human diseases. Physiol Rev 2007; 87:1377-1408.
10. Schubert U, Antón LC, Gibbs J, Norbury CC, Yewdell JW, Bennink JR. Rapid degradation of a large fraction of newly synthesized proteins by proteasomes. Nature 2000;404:770-774.
11. Vabulas RM, Hartl FU. Protein synthesis upon acute nutrient restriction relies on proteasome function. Science 2005;310: 1960-1963.
12. Gidalevitz T, Prahlad V, Morimoto RI. The stress of protein misfolding: from single cells to multicellular organisms. Cold Spring Harb Perspect Biol 2011;3:3.
13. Sridhar S, Botbol Y, Macian F, Cuervo AM. Autophagy and disease: always two sides to a problem. J Pathol 2012;226:255-273.
14. Lindquist SL, Kelly JW. Chemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosis. Cold Spring Harb Perspect Biol 2011;3:3.
15. Back SH, Kaufman RJ. Endoplasmic reticulum stress and type 2 diabetes. Annu Rev Biochem 2012;81:767-793.
16. Morimoto RI. The heat shock response: systems biology of proteotoxic stress in aging and disease. Cold Spring Harb Symp Quant Biol 2011;76:91-99.
17. Powers ET, Morimoto RI, Dillin A, Kelly JW, Balch WE. Biological and chemical approaches to diseases of proteostasis deficiency. Annu Rev Biochem 2009;78:959-991.
18. Haynes CM, Ron D. The mitochondrial UPR-protecting organelle protein homeostasis. J Cell Sci 2010;123:3849-3855.
19. Margittai E, Sitia R. Oxidative protein folding in the secretory pathway and redox signaling across compartments and cells. Traffic 2011;12: 1-8.
20. Walter P, Ron D. The unfolded protein response: from stress pathway to homeostatic regulation. Science 2011;334:1081-1086.
21. Bouchecareilh M, Hutt DM, Szajner P, Flotte TR, Balch WE. Histone deacetylase inhibitor (HDACi) suberoylanilide hydroxamic acid (SAHA)-mediated correction of α1-antitrypsin deficiency. J Biol Chem 2012;287:38265-38278.
22. Trüe O, Matthias P. Interplay between histone deacetylases and autophagy-from cancer therapy to neurodegeneration. Immunol Cell Biol 2012;90:78-84.
23. Finley D. Recognition and processing of ubiquitin-protein conjugates by the proteasome. Annu Rev Biochem 2009;78:477-513.
24. Bouchecareilh M, Balch WE. Proteostasis, an emerging therapeutic paradigm for managing inflammatory airway stress disease. Curr Mol Med 2012;12:815-826.
25. Bodas M, Tran I, Vij N. Therapeutic strategies to correct proteostasisimbalance in chronic obstructive lung diseases. Curr Mol Med 2012; 12:807-814.
26. Weathington NM, Sznajder JI, Mallampalli RK. The emerging role of the ubiquitin proteasome in pulmonary biology and disease. Am J Respir Crit Care Med 2013;188:530-537.
27. Balch WE, Roth DM, Hutt DM. Emergent properties of proteostasis in managing cystic fibrosis. Cold Spring Harb Perspect Biol 2011;3:3.
28. Bouchecareilh M, Conkright JJ, Balch WE. Proteostasis strategies for restoring alpha1-antitrypsin deficiency. Proc Am Thorac Soc 2010;7: 415-422.
29. Ekeowa UI, Gooptu B, Belorgey D, Hägglöf P, Karlsson-Li S, Miranda E, Pérez J, MacLeod I, Kroger H, Marciniak SJ, et al. alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies. Clin Sci (Lond) 2009;116:837-850.
30. Lawson WE, Loyd JE, Degryse AL. Genetics in pulmonary fibrosis-familial cases provide clues to the pathogenesis of idiopathic pulmonary fibrosis. Am J Med Sci 2011;341:439-443.
31. Lawson WE, Grant SW, Ambrosini V, Womble KE, Dawson EP, Lane KB, Markin C, Renzoni E, Lympany P, Thomas AQ, et al. Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF. Thorax 2004;59:977-980.
32. Pierson DM, Ionescu D, Qing G, Yonan AM, Parkinson K, Colby TC, Leslie K. Pulmonary fibrosis in Hermansky-Pudlak syndrome: a case report and review. Respiration 2006;73:382-395.
33. Seibold MA, Wise AL, Speer MC, Steele MP, Brown KK, Loyd JE, Fingerlin TE, Zhang W, Gudmundsson G, Groshong SD, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011;364:1503-1512.
34. Henke MO, John G, Germann M, Lindemann H, Rubin BK. MUC5AC and MUC5B mucins increase in cystic fibrosis airway secretions during pulmonary exacerbation. Am J Respir Crit Care Med 2007; 175:816-821.
35. Kirkham S, Kolsum U, Rousseau K, Singh D, Vestbo J, Thornton DJ. MUC5B is the major mucin in the gel phase of sputum in chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2008; 178:1033-1039.
36. Fu W, O'Connor TD, Jun G, Kang HM, Abecasis G, Leal SM, Gabriel S, Rieder MJ, Altshuler D, Shendure J, et al.; NHLBI Exome Sequencing Project. Analysis of 6,515 exomes reveals the recent origin of most human protein-coding variants. Nature 2013;493:216-220.
37. Kirkham PA, Caramori G, Casolari P, Papi AA, Edwards M, Shamji B, Triantaphyllopoulos K, Hussain F, Pinart M, Khan Y, et al. Oxidative stress-induced antibodies to carbonyl-modified protein correlate with severity of chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2011;184:796-802.
38. Debigaré R, Côté CH, Maltais F. Ubiquitination and proteolysis in limb and respiratory muscles of patients with chronic obstructive pulmonary disease. Proc Am Thorac Soc 2010;7:84-90.
39. Ward PA. Oxidative stress: acute and progressive lung injury. Ann N Y Acad Sci 2010;1203:53-59.
40. Weber-Carstens S, Schneider J, Wollersheim T, Assmann A, Bierbrauer J, Marg A, Al Hasani H, Chadt A, Wenzel K, Koch S, et al. Critical illness myopathy and GLUT4: significance of insulin and muscle contraction. Am J Respir Crit Care Med 2013;187:387-396.
41. Jaber S, Petrof BJ, Jung B, Chanques G, Berthet JP, Rabuel C, Bouyabrine H, Courouble P, Koechlin-Ramonatxo C, Sebbane M, et al. Rapidly progressive diaphragmatic weakness and injury during mechanical ventilation in humans. Am J Respir Crit Care Med 2011;183: 364-371.
42. Fermoselle C, Rabinovich R, Ausín P, Puig-Vilanova E, Coronell C, Sanchez F, Roca J, Gea J, Barreiro E. Does oxidative stress modulate limb muscle atrophy in severe COPD patients? Eur Respir J 2012;40: 851-862.
43. Helliwell TR, Wilkinson A, Griffiths RD, McClelland P, Palmer TE, Bone JM. Muscle fibre atrophy in critically ill patients is associated with the loss of myosin filaments and the presence of lysosomal enzymes and ubiquitin. Neuropathol Appl Neurobiol 1998;24:507-517.
44. Mizumura K, Cloonan SM, Haspel JA, Choi AM. The emerging importance of autophagy in pulmonary diseases. Chest 2012;142: 1289-1299.
45. Min T, Bodas M, Mazur S, Vij N. Critical role of proteostasis-imbalance in pathogenesis of COPD and severe emphysema. J Mol Med (Berl) 2011;89:577-593.
46. Korfei M, Ruppert C, Mahavadi P, Henneke I, Markart P, Koch M, Lang G, Fink L, Bohle RM, Seeger W, et al. Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008;178:838-846.
47. Sutendra G, Dromparis P, Wright P, Bonnet S, Haromy A, Hao Z, McMurtry MS, Michalak M, Vance JE, Sessa WC, et al. The role of Nogo and the mitochondria-endoplasmic reticulum unit in pulmonary hypertension. Sci Transl Med 2011;3:88ra55.
48. Nerelius C, Martin E, Peng S, Gustafsson M, Nordling K, Weaver T, Johansson J. Mutations linked to interstitial lung disease can abrogate anti-amyloid function of prosurfactant protein C. Biochem J 2008;416:201-209.
49. Kenche H, Baty CJ, Vedagiri K, Shapiro SD, Blumental-Perry A. Cigarette smoking affects oxidative protein folding in endoplasmic reticulum by modifying protein disulfide isomerase. FASEB J 2013; 27:965-977.
50. Barreiro E, Peinado VI, Galdiz JB, Ferrer E, Marin-Corral J, Sánchez F, Gea J, Barberà JA; ENIGMA in COPD Project. Cigarette smokeinduced oxidative stress: A role in chronic obstructive pulmonary disease skeletal muscle dysfunction. Am J Respir Crit Care Med 2010;182:477-488.
51. Naidoo N. Roles of endoplasmic reticulum and energetic stress in disturbed sleep. Neuromolecular Med 2012;14:213-219.
52. Li Y, Veasey SC. Neurobiology and neuropathophysiology of obstructive sleep apnea. Neuromolecular Med 2012;14:168-179.
53. Naidoo N, Zhu J, Zhu Y, Fenik P, Lian J, Galante R, Veasey S. Endoplasmic reticulum stress in wake-active neurons progresses with aging. Aging Cell 2011;10:640-649.
54. Weibel ER. What makes a good lung? Swiss Med Wkly 2009;139: 375-386.
55. McKone EF, Goss CH, Aitken ML. CFTR genotype as a predictor of prognosis in cystic fibrosis. Chest 2006;130:1441-1447.
56. American Thoracic Society; European Respiratory Society. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 2003;168: 818-900.
57. Vestbo J, Hurd SS, Agustí AG, Jones PW, Vogelmeier C, Anzueto A, Barnes PJ, Fabbri LM, Martinez FJ, Nishimura M, et al. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Am J Respir Crit Care Med 2013;187:347-365.
58. Van Goor F, Hadida S, Grootenhuis PDJ, Burton B, Stack JH, Straley KS, Decker CJ, Miller M, McCartney J, Olson ER, et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci USA 2011;108:18843-18848.
59. Treatment with VX-661 and ivacaftor in a phase 2 study resulted in statistically significant improvements in lung function in people with cystic fibrosis who have two copies of the F508del mutation. 2013 [accessed 2013 Aug 8]. Available from: http://investors.vrtx.com/releasedetail.cfm?ReleaseID=757597
60. Sloane PA, Shastry S, Wilhelm A, Courville C, Tang LP, Backer K, Levin E, Raju SV, Li Y, Mazur M, et al. A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease. PLoS ONE 2012;7: e39809.
61. Yao H, Rahman I. Role of histone deacetylase 2 in epigenetics and cellular senescence: implications in lung inflammaging and COPD. Am J Physiol Lung Cell Mol Physiol 2012;303:L557-L566.
62. Mutlu GM, Budinger GRS, Wu M, Lam AP, Zirk A, Rivera S, Urich D, Chiarella SE, Go LHT, Ghosh AK, et al. Proteasomal inhibition after injury prevents fibrosis by modulating TGF-β(1) signalling. Thorax 2012;67:139-146.
63. San Miguel JF, Schlag R, Khuageva NK, Dimopoulos MA, Shpilberg O, Kropff M, Spicka I, Petrucci MT, Palumbo A, Samoilova OS, et al.; VISTA Trial Investigators. Bortezomib plus melphalan and prednisone for initial treatment of multiple myeloma. N Engl J Med 2008;359: 906-917.
64. Chan JCY, Piper DE, Cao Q, Liu D, King C, Wang W, Tang J, Liu Q, Higbee J, Xia Z, et al. A proprotein convertase subtilisin/kexin type 9 neutralizing antibody reduces serum cholesterol in mice and nonhuman primates. Proc Natl Acad Sci U S A 2009;106:9820-9825.
65. Stein EA, Mellis S, Yancopoulos GD, Stahl N, Logan D, Smith WB, Lisbon E, Gutierrez M, Webb C, Wu R, et al. Effect of a monoclonal antibody to PCSK9 on LDL cholesterol. N Engl J Med 2012;366: 1108-1118.