Altered biogenesis of Δf508-CFTR following treatment with doxorubicin

Cellular Physiology and Biochemistry

Volumn 20, Issue 5, 2007, Pages 465-472

  Maitra, Rangan ^a   Hamilton, Joshua W ^b  

^a RTI INTERNATIONAL   (United States)

^b DARTMOUTH MEDICAL SCHOOL   (United States)

Author keywords

Anthracyclines; Biogenesis; CFTR; Doxorubicin; Pharmacology; F508

Indexed keywords

CHAPERONE; DOXORUBICIN; HEAT SHOCK PROTEIN 70; TRANSMEMBRANE CONDUCTANCE REGULATOR; TRYPSIN; PEPTIDE HYDROLASE; PHENYLALANINE; UBIQUITIN;

ANIMAL CELL; ARTICLE; BIOGENESIS; CELL STRAIN; CONTROLLED STUDY; HALF LIFE TIME; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN PROCESSING; ANIMAL; BIOSYNTHESIS; CELL LINE; DOG; GENE DELETION; GENETICS; METABOLISM; PROTEIN BINDING;

ANIMALS; CELL LINE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DOGS; DOXORUBICIN; HSP70 HEAT-SHOCK PROTEINS; PEPTIDE HYDROLASES; PHENYLALANINE; PROTEIN BINDING; SEQUENCE DELETION; UBIQUITIN;

EID: 34548370676     PISSN: 10158987     EISSN: None     Source Type: Journal    
DOI: 10.1159/000107530     Document Type: Article

References (53)

1. Turcios NL: Cystic fibrosis: an overview. J Clin Gastroenterol 2005;39:307-317.
2. Powell K, Zeitlin PL: Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting. Adv Drug Deliv Rev 2002;54:1395-1408.
3. Wang F, Zeltwanger S, Hu S, Hwang TC: Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels. J Physiol 2000;524:637-648.
4. Dalemans W, Barbry P, Champigny G, Jallat S, Dott K, Dreyer D, Crystal RG, Pavirani A, Lecocq JP, Lazdunski M: Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation. Nature 1991;354:526-528.
5. Kopito RR: Biosynthesis and degradation of CFTR. Physiol Rev 1999;79:S167-173.
6. Johnson LG, Olsen JC, Sarkadi B, Moore KL, Swanstrom R, Boucher RC: Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis. Nat Genet 1992;2:21-25.
7. Pedemonte N, Lukacs GL, Du K, Caci E, Zegarra-Moran O, Galietta LJ, Verkman AS: Small-molecule correctors of defective ΔF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest 2005;115:2564-2571.
8. Van Goor F, Straley KS, Cao D, Gonzalez J, Hadida S, Hazlewood A, Joubran J, Knapp T, Makings LR, Miller M, Neuberger T, Olson E, Panchenko V, Rader J, Singh A, Stack JH, Tung R, Grootenhuis PD, Negulescu P: Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 2006;290:L1117-1130.
9. Loo TW, Bartlett MC, Clarke DM: Rescue of ΔF508 and other misprocessed CFTR mutants by a novel quinazoline compound. Mol Pharm 2005;2:407-413.
10. Klein I, Sarkadi B, Varadi A: An inventory of the human ABC proteins. Biochim Biophys Acta 1999;1461:237-262.
11. Johannesson M, Nordqvist AC, Bogdanovic N, Hjelte L, Schalling M: Polymorphic expression of multidrug resistance mRNA in lung parenchyma of nonpregnant and pregnant rats: a comparison to cystic fibrosis mRNA expression. Biochem Biophys Res Commun 1997;239:606-611.
12. Trezise AE, Ratcliff R, Hawkins TE, Evans MJ, Freeman TC, Romano PR, Higgins CF, Colledge WH: Co-ordinate regulation of the cystic fibrosis and multidrug resistance genes in cystic fibrosis knockout mice. Hum Mol Genet 1997;6:527-537.
13. Trezise AE, Romano PR, Gill DR, Hyde SC, Sepulveda FV, Buchwald M, Higgins CF: The multidrug resistance and cystic fibrosis genes have complementary patterns of epithelial expression. EMBO J 1992;11:4291-4303.
14. Maitra R, Hamilton JW: Arsenite regulates Cystic Fibrosis Transmembrane Conductance Regulator and P-glycoprotein: evidence of pathway independence. Cell Physiol Biochem 2005;16:109-118.
15. Maitra R, Shaw CM, Stanton BA, Hamilton JW: Increased functional cell surface expression of CFTR and ΔF508-CFTR by the anthracycline doxorubicin. Am J Physiol Cell Physiol 2001;280:C1031-1037.
16. Maitra R, Shaw CM, Stanton BA, Hamilton JW: Functional Enhancement of CFTR Expression by Mitomycin C. Cell Physiol Biochem 2001;11:93-98.
17. Moyer BD, Stanton BA: Analysis of CFTR trafficking and polarization using green fluorescent protein and confocal microscopy. Methods Mol Med 2002;70:217-227.
18. Kartner N, Riordan JR: Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator. Methods Enzymol 1998;292:629-652.
19. Kalin N, Claass A, Sommer M, Puchelle E, Tummler B: DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis. J Clin Invest 1999;103:1379-1389.
20. Yang Y, Janich S, Cohn JA, Wilson JM: The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment. Proc Natl Acad Sci U S A 1993;90:9480-9484.
21. Johnston JA, Ward CL, Kopito RR: Aggresomes: a cellular response to misfolded proteins. J Cell Biol 1998;143:1883-1898.
22. Ward CL, Omura S, Kopito RR: Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 1995;83:121-127.
23. Gelman MS, Kopito RR: Cystic fibrosis: premature degradation of mutant proteins as a molecular disease mechanism. Methods Mol Biol 2003;232:27-37.
24. Chen EY, Bartlett MC, Clarke DM: Cystic fibrosis transmembrane conductance regulator has an altered structure when its maturation is inhibited. Biochemistry 2000;39:3797-3803.
25. Loo TW, Clarke DM: Superfolding of the partially unfolded core-glycosylated intermediate of human P-glycoprotein into the mature enzyme is promoted by substrate-induced transmembrane domain interactions. J Biol Chem 1998;273:14671-14674.
26. Sato S, Ward CL, Kopito RR: Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro. J Biol Chem 1998;273:7189-7192.
27. Ward CL, Kopito RR: Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins. J Biol Chem 1994;269:25710-25718.
28. Younger JM, Ren HY, Chen L, Fan CY, Fields A, Patterson C, Cyr DM: A foldable ΔF508-CFTR biogenic intermediate accumulates upon inhibition of the Hsc70-CHIP E3 ubiquitin ligase. J Cell Biol 2004;167:1075-1085.
29. Wang S, Konorev EA, Kotamraju S, Joseph J, Kalivendi S, Kalyanaraman B: Doxorubicin induces apoptosis in normal and tumor cells via distinctly different mechanisms. intermediacy of H(2)O(2)- and p53-dependent pathways. J Biol Chem 2004;279:25535-25543.
30. Sharma M, Pampinella F, Nemes C, Benharouga M, So J, Du K, Bache KG, Papsin B, Zerangue N, Stenmark H, Lukacs GL: Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes. J Cell Biol 2004;164:923-933.
31. Benharouga M, Sharma M, Lukacs GL: CFTR folding and maturation in cells. Methods Mol Med 2002;70:229-243.
32. Sharma M, Benharouga M, Hu W, Lukacs GL: Conformational and temperature-sensitive stability defects of the ΔF508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments. J Biol Chem 2001;276:8942-8950.
33. Zhang Y, Nijbroek G, Sullivan ML, McCracken AA, Watkins SC, Michaelis S, Brodsky JL: Hsp70 molecular chaperone facilitates endoplasmic reticulum-associated protein degradation of cystic fibrosis transmembrane conductance regulator in yeast. Mol Biol Cell 2001;12:1303-1314.
34. Jensen TJ, Loo MA, Pind S, Williams DB, Goldberg AL, Riordan JR: Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 1995;83:129-135.
35. Chang XB, Kartner N, Seibert FS, Aleksandrov AA, Kloser AW, Kiser GL, Riordan JR: Heterologous expression systems for study of cystic fibrosis transmembrane conductance regulator. Methods Enzymol 1998;292:616-629.
36. Huber SA: Heat-shock protein induction in adriamycin and picornavirus-infected cardiocytes. Lab Invest 1992;67:218-224.
37. Meacham GC, Lu Z, King S, Sorscher E, Tousson A, Cyr DM: The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis. EMBO J 1999;18:1492-1505.
38. Choo-Kang LR, Zeitlin PL: Induction of HSP70 promotes ΔF508 CFTR trafficking. Am J Physiol Lung Cell Mol Physiol 2001;281:L58-68.
39. Qu BH, Strickland E, Thomas PJ: Cystic fibrosis: a disease of altered protein folding. Journal of Bioenergetics & Biomembranes 1997;29:483-490.
40. Loo TW, Bartlett MC, Clarke DM: Rescue of folding defects in ABC transporters using pharmacological chaperones. J Bioenerg Biomembr 2005;37:501-507.
41. Yang H, Shelat AA, Guy RK, Gopinath VS, Ma T, Du K, Lukacs GL, Taddei A, Folli C, Pedemonte N, Galietta LJ, Verkman AS: Nanomolar affinity small molecule correctors of defective ΔF508-CFTR chloride channel gating. J Biol Chem 2003;278:35079-35085.
42. Wang Y, Loo TW, Bartlett MC, Clarke DM: Modulating the folding of P-glycoprotein and cystic fibrosis transmembrane conductance regulator truncation mutants with pharmacological chaperones. Mol Pharmacol 2007;71:751-758.
43. Tagliavini F, McArthur RA, Canciani B, Giaccone G, Porro M, Bugiani M, Lievens PM, Bugiani O, Peri E, Dall'Ara P, Rocchi M, Poli G, Forloni G, Bandiera T, Varasi M, Suarato A, Cassutti P, Cervini MA, Lansen J, Salmona M, Post C: Effectiveness of anthracycline against experimental prion disease in Syrian hamsters. Science 1997;276:1119-1122.
44. Gianni L, Bellotti V, Gianni AM, Merlini G: New drug therapy of amyloidoses: resorption of AL-type deposits with 4′-iodo-4′- deoxydoxorubicin. Blood 1995;86:855-861.
45. Amaral MD: CFTR and chaperones: processing and degradation. J Mol Neurosci 2004;23:41-48.
46. Lyakhovich A, Shekhar MP: Supramolecular complex formation between Rad6 and proteins of the p53 pathway during DNA damage-induced response. Mol Cell Biol 2003;23:2463-2475.
47. Jiang C, Fang SL, Xiao YF, O'Connor SP, Nadler SG, Lee DW, Jefferson DM, Kaplan JM, Smith AE, Cheng SH: Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF508 cells by deoxyspergualin. Am J Physiol 1998;275:C171-178.
48. Egan ME, Glockner-Pagel J, Ambrose C, Cahill PA, Pappoe L, Balamuth N, Cho E, Canny S, Wagner CA, Geibel J, Caplan MJ: Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells. Nat Med 2002;8:485-492.
49. Wang X, Venable J, LaPointe P, Hutt DM, Koulov AV, Coppinger J, Gurkan C, Kellner W, Matteson J, Plutner H, Riordan JR, Kelly JW, Yates JR, 3rd, Balch WE: Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 2006;127:803-815.
50. Dorin JR, Farley R, Webb S, Smith SN, Farini E, Delaney SJ, Wainwright BJ, Alton EW, Porteous DJ: A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction. Gene Ther 1996;3:797-801.
51. Gonsette RE: New immunosuppressants with potential implication in multiple sclerosis. J Neurol Sci 2004;223:87-93.
52. Gonsette RE, Dubois B: Pixantrone (BBR2778): a new immunosuppressant in multiple sclerosis with a low cardiotoxicity. J Neurol Sci 2004;223:81-86.
53. Borchmann P, Reiser M: Pixantrone (Novuspharma). IDrugs 2003;6:486-490.