Exploring prion protein biology in flies: Genetics and beyond

Prion

Volumn 4, Issue 1, 2010, Pages

  Rincon Limas, Diego E ^a   Casas Tinto, Sergio ^b   Fernandez Funez, Pedro ^c  

^a University of Texas Medical Branch School of Medicine   (United States)

^b Structural Biology and Biocomputing Programme   (Spain)

^c UNIVERSITY OF FLORIDA   (United States)

Author keywords

Drosophila model; Genetics; Neurodegeneration; Prion protein; Spontaneous misfolding

Indexed keywords

ALPHA SYNUCLEIN; ATAXIN 1; ATAXIN 3; HEAT SHOCK PROTEIN 70; POLYGLUTAMINE; PRION PROTEIN; TRANSCRIPTION FACTOR GAL4;

ALZHEIMER DISEASE; AMYOTROPHIC LATERAL SCLEROSIS; BEHAVIOR; CYTOLOGY; DEGENERATIVE DISEASE; DEVELOPMENTAL STAGE; DROSOPHILA MELANOGASTER; ENZYME ACTIVITY; FLY; GENE; GENE CONTROL; GENE EXPRESSION; GENE LOCATION; GENE OVEREXPRESSION; GENE SWITCHING; GENE TARGETING; GENETIC CODE; GENETIC ENGINEERING; GENETIC MANIPULATION; GENETIC STRAIN; GENOME; GENOMICS; IMMUNITY; IN VIVO STUDY; KENNEDY DISEASE; MACHADO JOSEPH DISEASE; MODEL; MOLECULAR BIOLOGY; MOLECULAR MODEL; MYOTONIC DYSTROPHY; NERVE DEGENERATION; NONHUMAN; NOTE; PARKINSON DISEASE; PATHOGENESIS; PRION; PROMOTER REGION; PROTEIN FOLDING; PROTEIN PHOSPHORYLATION; RNA INTERFERENCE; SIGNAL TRANSDUCTION; SPINOCEREBELLAR DEGENERATION; TAUOPATHY; TRANSCRIPTION INITIATION; TRANSGENE; ANIMAL; ARTICLE; GENETICS; METABOLISM;

ANIMALS; DROSOPHILA MELANOGASTER; NEURODEGENERATIVE DISEASES; PRIONS; PROTEIN FOLDING;

EID: 77949286922     PISSN: 19336896     EISSN: 1933690X     Source Type: Journal    
DOI: None     Document Type: Note

References (50)

1. Adams MD, Celniker SE, Holt RA, Evans CA, Gocayne JD, Amanatides PG, et al. The genome sequence of Drosophila melanogaster. Science 2000; 287:16-20.
2. Venter JC, Adams MD, Myers EW, Li PW, Mural RJ, Sutton GG, et al. The sequence of the human genome. Science 2001; 291:1304-51.
3. Lander ES, Linton LM, Birren B, Nusbaum C, Zody MC, Baldwin J, et al. Initial sequencing and analysis of the human genome. Nature 2001; 409:860-921.
4. Matthews KA, Kaufman TC, Gelbart WM. Research resources for Drosophila: the expanding universe. Nat Rev Genet 2005; 6:179-93.
5. Brand AH, Perrimon N. Targeted gene expression as a means of altering cell fates and generating dominant phenotypes. Development 1993; 118:401-15.
6. Rubin GM, Spradling AC. Genetic transformation of Drosophila with transposable element vectors. Science 1982; 218:348-53.
7. McGuire SE, Roman G, Davis RL. Gene expression systems in Drosophila: a synthesis of time and space. Trends Genet 2004; 20:384-91.
8. Stebbins MJ, Urlinger S, Byrne G, Bello B, Hillen W, Yin JC. Tetracycline-inducible systems for Drosophila. Proc Natl Acad Sci USA 2001; 98:10775-80.
9. McGuire SE, Le PT, Osborn AJ, Matsumoto K, Davis RL. Spatiotemporal rescue of memory dysfunction in Drosophila. Science 2003; 302:1765-8.
10. Venken KJ, Bellen HJ. Emerging technologies for gene manipulation in Drosophila melanogaster. Nat Rev Genet 2005; 6:167-78.
11. Ryder E, Ashburner M, Bautista-Llacer R, Drummond J, Webster J, Johnson G, et al. The DrosDel deletion collection: a Drosophila genomewide chromosomal deficiency resource. Genetics 2007; 177:615-29.
12. Bellen HJ, Levis RW, Liao G, He Y, Carlson JW, Tsang G, et al. The BDGP gene disruption project: single transposon insertions associated with 40% of Drosophila genes. Genetics 2004; 167:761-81.
13. Fire A, Xu S, Montgomery MK, Kostas SA, Driver SE, Mello CC. Potent and specific genetic interference by double-stranded RNA in Caenorhabditis elegans. Nature 1998; 391:806-11.
14. Perrimon N, Mathey-Prevot B. Applications of high-throughput RNA interference screens to problems in cell and developmental biology. Genetics 2007; 175:7-16.
15. Venken KJ, Bellen HJ. Transgenesis upgrades for Drosophila melanogaster. Development 2007; 134:3571-84.
16. Warrick JM, Paulson HL, Gray-Board GL, Bui QT, Fischbeck KH, Pittman RN, et al. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell 1998; 93:939-49.
17. Jackson GR, Salecker I, Dong X, Yao X, Arnheim N, Faber PW, et al. Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. Neuron 1998; 21:633-42.
18. Fernandez-Funez P, Nino-Rosales ML, de Gouyon B, She WC, Luchak JM, Martinez P, et al. Identification of genes that modify ataxin-1-induced neurodegeneration. Nature 2000; 408:101-6.
19. Kazemi-Esfarjani P, Benzer S. Genetic suppression of polyglutamine toxicity in Drosophila. Science 2000; 287:1837-40.
20. Feany MB, Bender WW. A Drosophila model of Parkinson's disease. Nature 2000; 404:394-8.
21. Lu B, Vogel H. Drosophila models of neurodegenerative diseases. Annu Rev Pathol 2009; 4:315-42.
22. Bilen J, Bonini NM. Genome-wide screen for modifiers of ataxin-3 neurodegeneration in Drosophila. PLoS Genet 2007; 3:1950-64.
23. Cao W, Song HJ, Gangi T, Kelkar A, Antani I, Garza D, et al. Identification of novel genes that modify phenotypes induced by Alzheimer's beta-amyloid overexpression in Drosophila. Genetics 2008; 178:1457-71.
24. Chen HK, Fernandez-Funez P, Acevedo SF, Lam YC, Kaytor MD, Fernandez MH, et al. Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell 2003; 113:457-68.
25. Emamian ES, Kaytor MD, Duvick LA, Zu T, Tousey SK, Zoghbi HY, et al. Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Neuron 2003; 38:375-87.
26. Steffan JS, Agrawal N, Pallos J, Rockabrand E, Trotman LC, Slepko N, et al. SUMO modification of Huntingtin and Huntington's disease pathology. Science 2004; 304:100-4.
27. Berger Z, Ravikumar B, Menzies FM, Oroz LG, Underwood BR, Pangalos MN, et al. Rapamycin alleviates toxicity of different aggregate-prone proteins. Hum Mol Genet 2006; 15:433-42.
28. Ravikumar B, Vacher C, Berger Z, Davies JE, Luo S, Oroz LG, et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat Genet 2004; 36:585-95.
29. Raeber AJ, Muramoto T, Kornberg TB, Prusiner SB. Expression and targeting of Syrian hamster prion protein induced by heat shock in transgenic Drosophila melanogaster. Mech Dev 1995; 51:317-27.
30. Deleault NR, Dolph PJ, Feany MB, Cook ME, Nishina K, Harris DA, et al. Post-transcriptional suppression of pathogenic prion protein expression in Drosophila neurons. J Neurochem 2003; 85:1614-23.
31. Gavin BA, Dolph MJ, Deleault NR, Geoghegan JC, Khurana V, Feany MB, et al. Accelerated accumulation of misfolded prion protein and spongiform degeneration in a Drosophila model of Gerstmann-Straussler-Scheinker syndrome. J Neurosci 2006; 26:12408-14.
32. Branco J, Al-Ramahi I, Ukani L, Perez AM, Fernandez-Funez P, Rincon-Limas D, et al. Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum Mol Genet 2008; 17:376-90.
33. Fernandez-Funez P, Salinas J, Nino-Rosales L, Rincon-Limas DE. Novel Suppressors of Abeta Neurotoxicity. In: Hanin I, Windisch M, Poewe W, Fisher A, eds. New Trends in Alzheimer and Parkinson Disorders: ADPD 2007. Salzburg, Austria: Medimont 2007; 37-41.
34. Fernandez-Funez P, Casas-Tinto S, Zhang Y, Gomez-Velazquez M, Morales-Garza MA, Cepeda-Nieto AC, et al. In vivo generation of neurotoxic prion protein: role for hsp70 in accumulation of misfolded isoforms. PLoS Genet 2009; 5:1000507.
35. Baron GS, Wehrly K, Dorward DW, Chesebro B, Caughey B. Conversion of raft associated prion protein to the protease-resistant state requires insertion of PrP-res (PrP(Sc)) into contiguous membranes. EMBO J 2002; 21:1031-40.
36. Castilla J, Saa P, Hetz C, Soto C. In vitro generation of infectious scrapie prions. Cell 2005; 121:195-206.
37. Atarashi R, Moore RA, Sim VL, Hughson AG, Dorward DW, Onwubiko HA, et al. Ultrasensitive detection of scrapie prion protein using seeded conversion of recombinant prion protein. Nat Methods 2007; 4:645-50.
38. Kovacs GG, Kurucz I, Budka H, Adori C, Muller F, Acs P, et al. Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease. Neurobiol Dis 2001; 8:881-9.
39. Kenward N, Hope J, Landon M, Mayer RJ. Expression of polyubiquitin and heat-shock protein 70 genes increases in the later stages of disease progression in scrapie-infected mouse brain. J Neurochem 1994; 62:1870-7.
40. Steele AD, Hutter G, Jackson WS, Heppner FL, Borkowski AW, King OD, et al. Heat shock factor 1 regulates lifespan as distinct from disease onset in prion disease. Proc Natl Acad Sci USA 2008; 105:13626-31.
41. Rambold AS, Miesbauer M, Rapaport D, Bartke T, Baier M, Winklhofer KF, et al. Association of Bcl-2 with Misfolded Prion Protein Is Linked to the Toxic Potential of Cytosolic PrP. Mol Biol Cell 2006; 17:3356-68.
42. Muchowski PJ, Wacker JL. Modulation of neurodegeneration by molecular chaperones. Nat Rev Neurosci 2005; 6:11-22.
43. Goloubinoff P, Rios Pde L. The mechanism of Hsp70 chaperones: (entropic) pulling the models together. Trends Biochem Sci 2007; 32:372-80.
44. Broquet AH, Thomas G, Masliah J, Trugnan G, Bachelet M. Expression of the molecular chaperone Hsp70 in detergent-resistant microdomains correlates with its membrane delivery and release. J Biol Chem 2003; 278:21601-6.
45. Rikhvanov EG, Romanova NV, Chernoff YO. Chaperone effects on prion and nonprion aggregates. Prion 2007; 1:217-22.
46. Romanova NV, Chernoff YO. Hsp104 and prion propagation. Protein Pept Lett 2009; 16:598-605.
47. Westergard L, Christensen HM, Harris DA. The cellular prion protein (PrP(C)): its physiological function and role in disease. Biochim Biophys Acta 2007; 1772:629-44.
48. van der Kamp MW, Daggett V. The consequences of pathogenic mutations to the human prion protein. Protein Eng Des Sel 2009; 22:461-8.
49. van Rheede T, Smolenaars MM, Madsen O, de Jong WW. Molecular evolution of the mammalian prion protein. Mol Biol Evol 2003; 20:111-21.
50. Rongyan Z, Xianglong L, Lanhui L, Xiangyun L, Fujun F. Evolution and differentiation of the prion protein gene (PRNP) among species. J Hered 2008; 99:647-52.