Prions

Godoy, J. M.; Skacel, M.; Nicaretta, D.H.

doi:10.1590/S0004-282X1991000200001

Resumos

Os autores se propõem a revisar alguns aspectos básicos sobre os prions, alertando sobre a possível participação destes na etiologia de algumas enfermidades degenerativas do sistema nervoso.

The authors make a review of the prion biological properties calling special attention for the possible participation of these agents in some neurodegenerative disorders.

CONTENTS CONTEÚDO

Prions

Prions

J. M. Godoy^I; M. Skacel^II; D.H. Nicaretta^III

^IProfessor Adjunto de Neurologia - Universidade Estadual do Rio de Janeiro (UERJ)

^IIProfessor Auxiliar de Neurologia - Universidade Estadual do Rio de Janeiro (UERJ)

^IIIMédico Residente do Serviço de Neurologia - Universidade Estadual do Rio de Janeiro (UERJ)

RESUMO

Os autores se propõem a revisar alguns aspectos básicos sobre os prions, alertando sobre a possível participação destes na etiologia de algumas enfermidades degenerativas do sistema nervoso.

SUMMARY

The authors make a review of the prion biological properties calling special attention for the possible participation of these agents in some neurodegenerative disorders.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Dr. José Maurício Godoy - Hospital Universitário Pedro Ernesto, UERJ - Av. 28 de Setembro 77, 2º andar - 20551 Rio de Janeiro RJ - Brasil.

1. Adams DH, Caspary EA, Fields EJ. The incorporation of (3H) thymidine, (14C) orotic acid, (14C) uridine-diphosphoglucose and (14C) glucosamine into a postribosomal fraction of normal and scrapie-affected mouse brain and spleen. J Gen Virol 1969, 4:89.
2. Alpert T, Cramp WA, Haig DA, Clarke MC. Does the agent of scrapie replicate without nucleic acid? Nature 1967, 214:764.
3. Barry RA, Kent SBH, McKinley MP, Meyer RK, De Armond SJ, Hood LE, Prusiner SB. Scrapie and cellular prion proteins share polypeptide epitopes. J Infect Dis 1986, 153:848.
4. Barry RA, McKinley MP, Bendheim PE, Lewis GK, De Armond SJ, Prusiner SB. Antibodies to the scrapie protein decorate prion rods. J Immunol 1985, 135:603.
5. Barry RA, Prusiner SB. Monoclonal antibodies to the cellular and scrapie prion proteins. J Infect Dis 1986, 154:518.
6. Basler K, Oesch B, Scott M, Westway D, Wälchii M, Groth DF, McKinley MP, Prusiner SB, Weismann C. Scrapie and cellular PrP isoforms are enconded by the same cromossomal gene. Cell 1986, 43:47.
7. Bazan JF, Fletterick RJ, Prusiner SB. AIDS virus and scrapie protein genes. Nature 1987, 325:581.
8. Bendheim PK, Barry RA, De Armond SJ, Stites DP, Prusiner SB. Antibodies to a scrapie prion protein. Nature 1984, 310:418.
9. Bockman JM, Kingsbury DT, McKinley MP, Bendheim PE, Prusiner SB. Creutzfeldt-Jakob disease prion proteins in human brains. N Engl J Med 1985, 312:73.
10. Bockman JM, Prusiner SB, Tateishi J, Kingsbury DT. Immunoblotting of Creuzfeldt-Jakob disease prion protein : host species-specific epitopes. Ann Neurol 1987, 21:589.
11. Bolton DC, Meyer RK, Prusiner SB. Scrapie PrP 27-30 is a sialoglycoprotein. J Virol 1985, 53:596.
12. Bruce ME, Dickinson AG. Biological evidence that scrapie agent has an independent genome. J Gen Virol 1987, 68:79.
13. Chandler RL, Fisher J. Experimental transmission of scrapie to rats. Lancet 1963, 2:1165.
14. Chesebro B, Race R, Wehely K, Nishio J, Bloom M, Lechner D, Bergstrom S, Robbins K, Mayer L, Keith JM, Garon C, Haase A. Identification of scrapie prion protein-specific in m-RNA in scrapie infected and uninfected brain. Nature 1985, 315:331.
15. De Armond SJ, McKinley MP, Barry RA, Braunfeld MB, McColloch JR, Prusiner SB. Identification of prion amyloid filaments in scrapie-infected brain. Cell 1985, 41:221.
16. Gajdusek DC. Slow virus diseases of the central nervous system. Am J Clin Pathol 1971, 56:320.
17. Gajdusek DC. Unconventional viruses and the origin and disappearance of Kuru. Science 1977, 197:943.
18. Gajdusek DC, Gibbs CJ Jr, Alpers M. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature 1966, 209:794.
19. Gaidusek DC, Zigas V. Degenerative diseases of the central nervous system in New Guinea : the endemic occurence of «Kuru» in the native population. N Engl J Med 1957, 257:974.
20. Gibbs CJ Jr, Gajdusek DC. Creutzfeldt-Jakob disease (spongiform encephalopathy) : transmission to chimpanzee. Science 1968, 161:388.
21. Gibbs CJ Jr, Gajdusek DC. Infection as the etiology of spongiform encephalopathy (Creutzfeldt-Jakob disease). Science 1969, 165:1023.
22. Gibbs CJ Jr, Gaidusek DC. Transmission of scrapie to the Cynomolgus monkey (Macaca fascicularis). Nature 1972, 236:73.
23. Gibbs CJ Jr, Gajdusek DC. Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals. Science 1973, 182:67.
24. Gibbs CJ Jr, Gajdusek DC, Latarget R. Unusual resistence to ionizing radiation of viruses of Kuru, Creutzfeldt-Jakob disease and scrapie. Proc Natl Aca Sci USA 1978, 75:6268.
25. Goldwater PN, Synek BJL, Koelmeyer TD, Scott PJ. Structures resembling scrapie: associated fibrils in AIDS encephalopathy. Lancet 1985, 2:447.
26. Goldwater PN, Synek BJL, Koelmeyer TD, Scott PJ. Scrapie-associated fibrils and AIDS encephalopathy. Lancet 1985, 2:1300.
27. Hadlow WJ. Scrapie and Kuru. Lancet 1959, 2:289.
28. Hadlow WJ, Prusiner SB Kennedy RC. Brain tissue from persons dying of Creutzfeldt-Jakob disease causes scrapie-like encephalopathy in goats. Ann Neurol 1980, 8:628.
29. Hunter GD. Gibbons RA, Kimberlin RH, Millson GC. Further studies of the infectivity and stability of extracts and homogenates derived from scrapie affected mouse brains. J Comp Path 1969, 79:101.
30. Hunter GD, Millson GC. Glycoprotein biosynthesis in normal and scrapie affected mouse brain. J Comp Path 1973, 83:217.
31. Hunter GD, Millson GC. El agente del «scrapie» : posición actual acerca de sua naturaleza. In Waterson AP (ed) : Progresos en Virología Clínica. Barcelona : Marin, 1978, p 65.
32. Kingsbury DT, Kasper KC, Stites DP, Watson JD, Hogan RN, Prusiner SB. Genetic control of scrapie and Creutzfeldt-Jakob disease in mice. J Immunol 1983, 131:491.
33. Klatzo I, Gajdusek DC, Zigas V. Pathology of Kuru. Lab Invest 1959, 8:799.
34. Kretzschmar HA, Prusiner SB, Stowring LE, De Armond SJ. Scrapie prion proteins are synthesized in neuron. Am J Path 1986, 122:1.
35. Lampert P, Hooks CJ Jr, Gaidusek DC. Altered plasma membranes in experimental scrapie. Acta Neuropath 1971, 19:81.
36. Lehninger AL. Carboidratos : estrutura e função biológica. In Lehninger AL (ed) : Princípios de Bioquímica. São Paulo : Sarvier, 1986, p 204.
37. Mackenzie A, Wilson AM, Dennis PF. Further observations changes in scrapie mouse brain. J Comp Path 1968, 78:489.
38. Marsh RF, Kimberlin RH. Comparison of scrapie and transmissible mink encephalopathy in hamsters. J Infect Dis 1975, 131:104.
39. Marsh RF, Malone TG, Semancik JS, Lancaster WD, Hanson RP. Evidence for an essential DNA component in the scrapie agent. Nature 1978, 275 : 146.
40. Masters CL, Gajdusek DC, Gibbs CJ Jr. Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome. Brain 1981, 104:559.
41. Matthews WB. Slow Infections. In Kennedy PGE, Johnson RT (eds): Infections of the Nervous System. London : Butterworths, 1987, p 227.
42. McKinley MP, Masiarz FR Prusiner SB. Reversible chemical modification of the scrapie agent. Science 1981, 214:1259.
43. Merz PA, Rohwer RG, Kascask R, Wisniewski HM, Somerville RA, Gibbs CJ Jr, Gajdusek DC. Infection-specific particle from the unconventional slow virus diseases. Science 1984, 225:437.
44. Millson GC, Hunter GD, Kimberlin RH. An experimental examination of the scrapie agent in cell membrane mixtures. J Comp Path 1971, 81:255.
45. Narang HK, Asher DM, Gajdusek DC. Evidence that DNA is present in abnormal tubu-lofilamentous structures found in scrapie. Proc Natl Acad Sci USA 1988, 85:3575.
46. Oesch B, Westaway D, Wälchli M, McKinley MP, Kent SBH, Aebersold R, Barry RA, Temost P, Teplow DB, Hood LE, Prusiner SB, Weissmann C. A cellular gene encodes scrapie PrP 27-30 protein. Cell 1985, 40:735.
47. Price RW, Navia BA. Infections in AIDS and in other immunosuppressed patients. In Kennedy PGE, Johnson RT (ed) : Infections of the Nervous System. London : Butterworths, 1987, p 248.
48. Prusiner SB. On prions cause dementia: molecular studies of the scrapie agent. In Appel SH (ed) : Current Neurology, Vol 4. New York: Wiley, 1982, p 201.
49. Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982, 216:136.
50. Prusiner SB. Occasional notes: some speculations about prions, amyloid, and Alzheimer disease. N Engl J Med 1984, 310:661.
51. Prusiner SB. Prions and neurodegenerative diseases. N Engl J Med 1987, 317:1571.
52. Prusiner SB, Bolton DC, Groth DF, Bowman KA, Cochran SP, McKinley MP. Further purification and characterization of scrapie prions. Biochemistry 1982, 21:6942.
53. Prusiner SB, Groth DF. Molecular properties, partial purification, and assay by incubation period measurements of hamster scrapie agent. Biochemistry 1980, 19:4883.
54. Prusiner SB, Groth DF, Bildstein C. Electrophoretic properties of the scrapie agent in agarose gels. Proc Natl Acad Sci USA 1980, 77:2984.
55. Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE, Purification and structural studies of a major scrapie prion protein. Cell 1984, 38:127.
56. Prusiner SB, Groth DF, McKinley MP - Thiocyanate and hydroxyl ions inactivate the scrapie agent. Proc Natl Acad Sci USA 1981, 78:4606.
57. Prusiner SB, McKinley MP, Bowman KA. Seropie prions aggregate to form amyloid-like birefringent rods. Cell 1983, 35:349.
58. Prusiner SB, McKinley MP, Groth DF. Scrapie agent contains a hydrofobic protein. Proc Natl Acad Sci USA, 1981, 78:6675.
59. Semancick JS, Marsh RF, Geelen JLMC, Hanson RP. Properties of the scrapie agent: endomembrane complex from hamster brain. J Virol 1976, 18:693.
60. Sigurdsson B - Observations on three slow infections of sheep. Br Vet 1954, 110:225, 307, 341.
61. Sparkes RS, Simon M, Cohn VH - Assignment of the human and mouse prion protein genes to homologous chromosomes. Proc Natl Acad Sei USA 1986, 83:7358.
62. Tateishi J, Ohta M, Koga M. Transmission of chronic spongiform encephalopathy with Kuru plaques for humans to small rodents. Ann Neurol 1979, 5:581.
63. Zarranz JJ, Rivera-Pomar JM, Salisachs P. Kuru plaques in the brain of two cases with Creutzfeldt-Jakob disease. J Neurol Sci 1979, 43:291.

Datas de Publicação

Publicação nesta coleção
22 Fev 2011
Data do Fascículo
Jun 1991

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Adams DH, Caspary EA, Fields EJ. The incorporation of (3H) thymidine, (14C) orotic acid, (14C) uridine-diphosphoglucose and (14C) glucosamine into a postribosomal fraction of normal and scrapie-affected mouse brain and spleen. J Gen Virol 1969, 4:89.

[2] 2. Alpert T, Cramp WA, Haig DA, Clarke MC. Does the agent of scrapie replicate without nucleic acid? Nature 1967, 214:764.

[3] 3. Barry RA, Kent SBH, McKinley MP, Meyer RK, De Armond SJ, Hood LE, Prusiner SB. Scrapie and cellular prion proteins share polypeptide epitopes. J Infect Dis 1986, 153:848.

[4] 4. Barry RA, McKinley MP, Bendheim PE, Lewis GK, De Armond SJ, Prusiner SB. Antibodies to the scrapie protein decorate prion rods. J Immunol 1985, 135:603.

[5] 5. Barry RA, Prusiner SB. Monoclonal antibodies to the cellular and scrapie prion proteins. J Infect Dis 1986, 154:518.

[6] 6. Basler K, Oesch B, Scott M, Westway D, Wälchii M, Groth DF, McKinley MP, Prusiner SB, Weismann C. Scrapie and cellular PrP isoforms are enconded by the same cromossomal gene. Cell 1986, 43:47.

[7] 7. Bazan JF, Fletterick RJ, Prusiner SB. AIDS virus and scrapie protein genes. Nature 1987, 325:581.

[8] 8. Bendheim PK, Barry RA, De Armond SJ, Stites DP, Prusiner SB. Antibodies to a scrapie prion protein. Nature 1984, 310:418.

[9] 9. Bockman JM, Kingsbury DT, McKinley MP, Bendheim PE, Prusiner SB. Creutzfeldt-Jakob disease prion proteins in human brains. N Engl J Med 1985, 312:73.

[10] 10. Bockman JM, Prusiner SB, Tateishi J, Kingsbury DT. Immunoblotting of Creuzfeldt-Jakob disease prion protein : host species-specific epitopes. Ann Neurol 1987, 21:589.

[11] 11. Bolton DC, Meyer RK, Prusiner SB. Scrapie PrP 27-30 is a sialoglycoprotein. J Virol 1985, 53:596.

[12] 12. Bruce ME, Dickinson AG. Biological evidence that scrapie agent has an independent genome. J Gen Virol 1987, 68:79.

[13] 13. Chandler RL, Fisher J. Experimental transmission of scrapie to rats. Lancet 1963, 2:1165.

[14] 14. Chesebro B, Race R, Wehely K, Nishio J, Bloom M, Lechner D, Bergstrom S, Robbins K, Mayer L, Keith JM, Garon C, Haase A. Identification of scrapie prion protein-specific in m-RNA in scrapie infected and uninfected brain. Nature 1985, 315:331.

[15] 15. De Armond SJ, McKinley MP, Barry RA, Braunfeld MB, McColloch JR, Prusiner SB. Identification of prion amyloid filaments in scrapie-infected brain. Cell 1985, 41:221.

[16] 16. Gajdusek DC. Slow virus diseases of the central nervous system. Am J Clin Pathol 1971, 56:320.

[17] 17. Gajdusek DC. Unconventional viruses and the origin and disappearance of Kuru. Science 1977, 197:943.

[18] 18. Gajdusek DC, Gibbs CJ Jr, Alpers M. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature 1966, 209:794.

[19] 19. Gaidusek DC, Zigas V. Degenerative diseases of the central nervous system in New Guinea : the endemic occurence of «Kuru» in the native population. N Engl J Med 1957, 257:974.

[20] 20. Gibbs CJ Jr, Gajdusek DC. Creutzfeldt-Jakob disease (spongiform encephalopathy) : transmission to chimpanzee. Science 1968, 161:388.

[21] 21. Gibbs CJ Jr, Gajdusek DC. Infection as the etiology of spongiform encephalopathy (Creutzfeldt-Jakob disease). Science 1969, 165:1023.

[22] 22. Gibbs CJ Jr, Gaidusek DC. Transmission of scrapie to the Cynomolgus monkey (Macaca fascicularis). Nature 1972, 236:73.

[23] 23. Gibbs CJ Jr, Gajdusek DC. Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals. Science 1973, 182:67.

[24] 24. Gibbs CJ Jr, Gajdusek DC, Latarget R. Unusual resistence to ionizing radiation of viruses of Kuru, Creutzfeldt-Jakob disease and scrapie. Proc Natl Aca Sci USA 1978, 75:6268.

[25] 25. Goldwater PN, Synek BJL, Koelmeyer TD, Scott PJ. Structures resembling scrapie: associated fibrils in AIDS encephalopathy. Lancet 1985, 2:447.

[26] 26. Goldwater PN, Synek BJL, Koelmeyer TD, Scott PJ. Scrapie-associated fibrils and AIDS encephalopathy. Lancet 1985, 2:1300.

[27] 27. Hadlow WJ. Scrapie and Kuru. Lancet 1959, 2:289.

[28] 28. Hadlow WJ, Prusiner SB Kennedy RC. Brain tissue from persons dying of Creutzfeldt-Jakob disease causes scrapie-like encephalopathy in goats. Ann Neurol 1980, 8:628.

[29] 29. Hunter GD. Gibbons RA, Kimberlin RH, Millson GC. Further studies of the infectivity and stability of extracts and homogenates derived from scrapie affected mouse brains. J Comp Path 1969, 79:101.

[30] 30. Hunter GD, Millson GC. Glycoprotein biosynthesis in normal and scrapie affected mouse brain. J Comp Path 1973, 83:217.

[31] 31. Hunter GD, Millson GC. El agente del «scrapie» : posición actual acerca de sua naturaleza. In Waterson AP (ed) : Progresos en Virología Clínica. Barcelona : Marin, 1978, p 65.

[32] 32. Kingsbury DT, Kasper KC, Stites DP, Watson JD, Hogan RN, Prusiner SB. Genetic control of scrapie and Creutzfeldt-Jakob disease in mice. J Immunol 1983, 131:491.

[33] 33. Klatzo I, Gajdusek DC, Zigas V. Pathology of Kuru. Lab Invest 1959, 8:799.

[34] 34. Kretzschmar HA, Prusiner SB, Stowring LE, De Armond SJ. Scrapie prion proteins are synthesized in neuron. Am J Path 1986, 122:1.

[35] 35. Lampert P, Hooks CJ Jr, Gaidusek DC. Altered plasma membranes in experimental scrapie. Acta Neuropath 1971, 19:81.

[36] 36. Lehninger AL. Carboidratos : estrutura e função biológica. In Lehninger AL (ed) : Princípios de Bioquímica. São Paulo : Sarvier, 1986, p 204.

[37] 37. Mackenzie A, Wilson AM, Dennis PF. Further observations changes in scrapie mouse brain. J Comp Path 1968, 78:489.

[38] 38. Marsh RF, Kimberlin RH. Comparison of scrapie and transmissible mink encephalopathy in hamsters. J Infect Dis 1975, 131:104.

[39] 39. Marsh RF, Malone TG, Semancik JS, Lancaster WD, Hanson RP. Evidence for an essential DNA component in the scrapie agent. Nature 1978, 275 : 146.

[40] 40. Masters CL, Gajdusek DC, Gibbs CJ Jr. Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome. Brain 1981, 104:559.

[41] 41. Matthews WB. Slow Infections. In Kennedy PGE, Johnson RT (eds): Infections of the Nervous System. London : Butterworths, 1987, p 227.

[42] 42. McKinley MP, Masiarz FR Prusiner SB. Reversible chemical modification of the scrapie agent. Science 1981, 214:1259.

[43] 43. Merz PA, Rohwer RG, Kascask R, Wisniewski HM, Somerville RA, Gibbs CJ Jr, Gajdusek DC. Infection-specific particle from the unconventional slow virus diseases. Science 1984, 225:437.

[44] 44. Millson GC, Hunter GD, Kimberlin RH. An experimental examination of the scrapie agent in cell membrane mixtures. J Comp Path 1971, 81:255.

[45] 45. Narang HK, Asher DM, Gajdusek DC. Evidence that DNA is present in abnormal tubu-lofilamentous structures found in scrapie. Proc Natl Acad Sci USA 1988, 85:3575.

[46] 46. Oesch B, Westaway D, Wälchli M, McKinley MP, Kent SBH, Aebersold R, Barry RA, Temost P, Teplow DB, Hood LE, Prusiner SB, Weissmann C. A cellular gene encodes scrapie PrP 27-30 protein. Cell 1985, 40:735.

[47] 47. Price RW, Navia BA. Infections in AIDS and in other immunosuppressed patients. In Kennedy PGE, Johnson RT (ed) : Infections of the Nervous System. London : Butterworths, 1987, p 248.

[48] 48. Prusiner SB. On prions cause dementia: molecular studies of the scrapie agent. In Appel SH (ed) : Current Neurology, Vol 4. New York: Wiley, 1982, p 201.

[49] 49. Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982, 216:136.

[50] 50. Prusiner SB. Occasional notes: some speculations about prions, amyloid, and Alzheimer disease. N Engl J Med 1984, 310:661.

[51] 51. Prusiner SB. Prions and neurodegenerative diseases. N Engl J Med 1987, 317:1571.

[52] 52. Prusiner SB, Bolton DC, Groth DF, Bowman KA, Cochran SP, McKinley MP. Further purification and characterization of scrapie prions. Biochemistry 1982, 21:6942.

[53] 53. Prusiner SB, Groth DF. Molecular properties, partial purification, and assay by incubation period measurements of hamster scrapie agent. Biochemistry 1980, 19:4883.

[54] 54. Prusiner SB, Groth DF, Bildstein C. Electrophoretic properties of the scrapie agent in agarose gels. Proc Natl Acad Sci USA 1980, 77:2984.

[55] 55. Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE, Purification and structural studies of a major scrapie prion protein. Cell 1984, 38:127.

[56] 56. Prusiner SB, Groth DF, McKinley MP - Thiocyanate and hydroxyl ions inactivate the scrapie agent. Proc Natl Acad Sci USA 1981, 78:4606.

[57] 57. Prusiner SB, McKinley MP, Bowman KA. Seropie prions aggregate to form amyloid-like birefringent rods. Cell 1983, 35:349.

[58] 58. Prusiner SB, McKinley MP, Groth DF. Scrapie agent contains a hydrofobic protein. Proc Natl Acad Sci USA, 1981, 78:6675.

[59] 59. Semancick JS, Marsh RF, Geelen JLMC, Hanson RP. Properties of the scrapie agent: endomembrane complex from hamster brain. J Virol 1976, 18:693.

[60] 60. Sigurdsson B - Observations on three slow infections of sheep. Br Vet 1954, 110:225, 307, 341.

[61] 61. Sparkes RS, Simon M, Cohn VH - Assignment of the human and mouse prion protein genes to homologous chromosomes. Proc Natl Acad Sei USA 1986, 83:7358.

[62] 62. Tateishi J, Ohta M, Koga M. Transmission of chronic spongiform encephalopathy with Kuru plaques for humans to small rodents. Ann Neurol 1979, 5:581.

[63] 63. Zarranz JJ, Rivera-Pomar JM, Salisachs P. Kuru plaques in the brain of two cases with Creutzfeldt-Jakob disease. J Neurol Sci 1979, 43:291.