Prion diseases of man and animal


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Abstract

Summarized in the paper are data on the molecular bases of transmissible spongoid encephalopathy (TSE), its
etiology, formation mechanism of prion proteins, STE-related strains, specific barriers to TSE, its pathogenesis
and treatment.

References

  1. Alper Т., Cramp W. A., Haig D. A., Clarke M. С. Does the agent of scrapie replicate without nucleic acid // Nature. - 1967. - Vol. 214. - P. 764-764.
  2. Anderson R. M., Donnelly С. A., Ferguson N. M. et al. Transmission dynamic and epidemiology of BSE in British cattle // Nature. - 1996. - Vol. 382. - P. 779-788.
  3. Antoine N., Cesborn J. Y., Coumans B. et al. Differential expression of cellular prion protein on human blood and tonsil lymphocytes // Haematology. - 2000. - Vol. 85. - P. 475-480.
  4. Aucouturier P., Geissmann F., Damott D. et al. Infected splenic dendritic cells are sufficient for prion transmission to the CNS in mouse scrapie // J. Clin. Invest. - 2001. - Vol. 108. - P. 703-708.
  5. Baron G. S., Wehrly K., Dorward D. V. et al. Conversion of raft associated prion protein the protease-resistant state requires insertion of PrP into continuous membranes// EMBO J. - 2002. -Vol.21. - P . 1031-1040.
  6. Bartz I. C., Kincaid A. E., Bessen R. A. Rapid prion neuroinvasion following tongue infection // J. Virol. - 2003. - Vol. 77. - P. 583-591.
  7. Bennion B. I., Daggett V. Protein conformation and diagnostic tests: the prion protein // Clin. Chem. - 2002. - Vol. 48. - P. 2105-2114.
  8. Bodemer W. The use of monoclonal antibodies in human prion disease // Naturwissenchaften. - 1999. - Bd 86. - S. 212- 220.
  9. Brown D. R., Hafiz F., Glassmith L. L. et al. Consequence of manganese replacement of Cu for prion protein function and proteinase resistance // EMBO J. - 2000. - Vol. 19. - P. 1180-1186.
  10. Brown D. R. Prion and prejudice normal protein and the synapse // Trends in Neurosc. - 2001. - Vol. 524. - P. 85-90.
  11. Brown D. Copper and prion disease // Brain Res. Bull. - 2000. - Vol. 55. - P. 165-173.
  12. Brown D. R., Hafiz F., Glassmith L. L. et al. Consequence of manganese replacement of copper for prion protein function and proteinase resistance // EMBO J. - 2000. - Vol. 19. - P. 1180-1186.
  13. Bueler H., Fisher M., Lang Y. et al. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein // Nature. - 1992. - Vol. 356. - P. 577-582.
  14. Caughy В., Chesebro B. Prion protein and the transmissible spongoform encephalopathies // Trend Cell Biol. - 1997. - Vol. 7. - P. 56-62.
  15. Chiarini L. В., Freitas A. R. O., Zanata S. M. et al. Cellular prior protein transduces new protective signal // EMBO J. - 2002. - Vol. 21. - P. 3317-3326.
  16. Chiesa R. P., Piccardo В., Chetti B. et al. Neurological illness in transgenic mice expressing a prior protein with an insertional mutation // Neuron. - 1998. - Vol. 21. - P. 1339-1351;
  17. Cohel G., Grigoriev V., Escaig-Haye F. et al. Ultrastructural localization of PPc at neuromuscular junction // J. Neurosci. Res. - 1999. - Vol. 55. - P. 261-267.
  18. Coitinho A. S., Roesler R., Martins V. R. et al. Cellular prion protein ablation impairs behavior as a function of age // Neuroreport. -2003. - Vol. 14. - P. 1375-1379.
  19. Collinge J. et al. Transmission of fatal familial insomnia to laboratory animals // Lancet. - 1995. - Vol. 346. - P. 569-570.
  20. DeArmond S. J., Prusiner S. B. Etiology and pathogenesis of prion diseases // Am. J. Pathol. - 1995. - Vol. 146. - P. 785- 811.
  21. Demaimay R., Adjou K., Lasmezas С. et al. Pharmacological studies of a new derivative of Amb. MS-8209 in mouse and humster scrapie // J. Gen. Virol. - 1994. - Vol. 75. - P. 2499-2503.
  22. Foumier I.-G., Escaig-Haye F., Grigoriev V. Ultrastructural localization of prion protein: physiological and pathological implications // Microsc. Res. Tech. - 2001. - Vol. 50. - P. 76- 88.
  23. Foumier I.-G. Nonneuronal cellular prion protein // Int. Rev. Cytol. - 2001. - Vol. 208. - P. 121-160.
  24. Foumier I.-G., Grigoriev V. B. Prion diseases: contribution of high resolution immune morphology // J. Cell Mol. Med. - 2001. - Vol. 5. - P. 367-377.
  25. Gambetti P., Parchi P., Peterson R. B. et al. Fatal familial insomnia and familial CJD: clinical, pathological and molecular feature // Brain Pathol. - 1995. - Vol. 5. - P. 43-51.
  26. Gauczynski S., Peyrin Y. M., Haik S. et al. The 37kDa/67kDa laminin receptor act as the cell-surface receptor for the cellular prion protein // EMBO J. - 2001. - Vol. 20. - P. 5863- 5875.
  27. Gibbs С J., Bolis С L. Normal isoform amyloid protein (PrP) in brain of spawning salmon // Mol. Psychiatry. - 1997. - Vol. 2. - P. 146-147.
  28. Giese A., Kretzchmar H. A. Prion-induced neuronal damage - the mechanisms of neuronal destruction in the subacute spongiform encephalopathies // Curr. Top. Microbiol. Immunol. - 2001. - Vol. 253. - P. 203-217.
  29. Glatzel M., Aguzzi A. Peripheral pathogenesis of prion disease // Microb. and Infect. - 2000. - Vol. 2. - P. 613-619.
  30. Glatzel M., Heppner F. L., Albers К. М. et al. Sympathetic innervation of lymphoreticular organs is rate-limited for prion neuroinvasion // Neuron. - 2001. - Vol. 31. - P. 25-34.
  31. Griffith J. S. Self-replication scrapie // Nature. - 1967. - Vol. 215. - P. 1043-1044.
  32. Grigoriev V. В., Adjou К. Т., Sales N. et al. Effect of the polyene antibiotic derivate MS-8209 on the astrocyte lysosomal system of scrapie-infected hamsters // J. Mol. Neurosci. - 2002. - Vol. 18. - P. 271-291.
  33. Grigoriev V. В., Escaig-Haye F., Streichenberger N. et al. Submicroscopic immunodetection of PrP in the brain of a patient with a new variant of Creutzfeld-Jakob disease // Neurosci. Lett. - 1999. - Vol. 263. - P. 59-63.
  34. Haeberle A., Rubaut-Barasson C., Bombarde G. et al. Synaptic prion protein immunoreactivity in the rodent cerebellum // Microsc. Res. Tech. - 2000. - Vol. 50. - P. 60-77.
  35. Harris D. A., Lele P., Snider W. D. Localization mRNA for a chicken prion protein by in situ hybridization // Proc. Natl. Acad. Sci. USA. - 1993. - Vol. 90. - P. 4309-4313.
  36. Harris D. Cellular biology of prion protein // Clin. Microbiol. Rev. - 1999. - Vol. 2. - P. 429-444.
  37. Heppner F. L., Musaht C., Arrighi I. et al. Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies // Science. - 2001. - Vol. 294. - P . 178-182.
  38. Herms J., Tings Т., Gall S. et al. Evidence of presynaptic location and function of the prion protein // J. Neurosci. - 1999. - Vol. 19. - P. 8866-8875.
  39. Hill A. F, Zeidler M., Ironside J. et al. Diagnosis of nv CJD by tonsil biopsy // Lancet. - 1997. - Vol. 349. - P. 99.
  40. Hill A. F., Joines S., Linehan J. et al. Species-barrier-independent prion replication in apparently resistant species // Proc. Natl. Acad. Sci. USA. - 2000. - Vol. 97. - P. 10248-10253.
  41. Horiuchi M., Priola S. A., Chabry J. et al. Interaction between heterologous form of prion protein. Binding, inhibition of conversion, and species barries // Proc. Natl. Acad. Sci. USA. - 2000. - Vol. 97. - P. 5836-5841.
  42. Ironside I. W., Head M. W. Variant CID disease and its transmission by blood // J. Thrombos. Haemostas. - 2003. - Vol. 1. - P. 1479-1486.
  43. Jackson G. S., Callinge J. The molecular pathology CJD: old and new variants // J. Clin. Pathol. - 2001. - Vol. 54. - P. 393-399.
  44. Klohn P. C, Stoltze L., FlechsigE. et al. A quantitative, highly sensitive cell-based infectivity assay for mouse scrapie prions // Proc. Natl. Acad. Sci. USA. - 2003. - Vol. 100. - P. 11666-11671.
  45. Korth C., May B. С. H., Cohen F. E. et al. Acridin and phenothiazin derivates as pharmatherapeutics for prion diseases // Proc. Natl. Acad. Sci. USA. - 2001. - Vol. 98. - P. 9836-9841.
  46. Korth C., Stierli В., Streit P. et al./ Prion (PrPsc) - specific epitope defined by a monoclonal antibody // Nature. - 1997. - Vol. 390. - P. 74-77.
  47. Kretzchmar H. A., Stowring L. E., Westway D. et al. Molecular cloning of a human prion protein cDNA // DNA. - 1986. - Vol. 5. - P. 315-324.
  48. Kuzuhara S., Kanazawa I., Sasaki H. et al. GSS disease // Ann. Neurol. - 1983. - Vol. 140. - P. 1411-1420
  49. Lasmezas C., Deslys J.-P., Robain O. et al. Transmission of the BSE to mice in the absence detectable abnormal prion protein // Science. - 1997. - Vol. 275. - P. 402-405.
  50. Lin Т., Li R., Pan T. et al. Intercellular transfer of the cellular prion protein // J. Biol. Chem. - 2002. - Vol. 277. - P. 47671-47678.
  51. Lotscher M., Recher M., Hunriker L. et al. Immunologically induced complement-dependent up-reulation of the prion protein in the mouse spleen: follicular dendritic cells versus capsule and trabeculae // J. Immunol. - 2003. - Vol. 170. - P. 6040- 6047.
  52. Mange A., Nishida N., Milhavet O. et al. Amb inhibits the generation of the scrapie isoform of the prion protein in infected cultures // J. Virol. - 2000. - Vol. 74. - P. 3135-3140.
  53. McBride P. A., Eikelenboom P., Kraal G. et al. PrP protein is associated with follicular dendritic cells of spleens and lymph nodes in uninfected and scrapie infected mice // J. Pathol. - 1992. - Vol. 168. - P. 413-418.
  54. McBride P. A., Beekes M. Pathological PrP is abundant in sympathic and sensory ganglia of hamsters fed with scrapie // Neurosci. Lett. - 1999. - Vol. 265. - P. 135-138.
  55. Mehlhom I., Groth D., Stochel J. et al. High level expression and characterization of purified 142-residue polypeptide of the prion protein // Biochemistry. - 1996. - Vol. 35. - P. 5528-5537.
  56. Miele G., Jeffrey M., Tumbull D. et al. Ablation of cellular prion protein expression affect mitohondrial number and morphology // Biochem. Biophys. Res. Commun. - 2002. - Vol. 291. - P. 372-377.
  57. Miele G., Manson I., Clinton M. A. A novel erythroid-specific marker of TSE // Nature Med. - 2001. - Vol. 7. - P. 361- 364.
  58. Mondjou M., Flobert Y., Grassi J. et al. Cellular prion protein status in sheep: tissue-specific biochemical signature // J. Gen. Virol. - 2001. - Vol. 82. - P. 2017-2024.
  59. Montrasio F., Frigg R., Glatzel M. et al. Impaired prion replication in spleen of mice lacking functional follicular dendritic cells // Science. - 2000. - Vol. 288. - P. 1257-1259.
  60. Moore R. C., Lee J. J., Silverman G. L. et al. Ataxia in PrP-deficient mice is associated with upregulation of the novel PrPlike protein Doppel // J. Mol. Biol. - 1999. - Vol. 292. - P. 797-817.
  61. Muniz M., Riezman H. Intracellular transport of GPI-anchored proteins // EMBO J. - 2000. - Vol. 19. - P. 10-15.
  62. Palmer M. S., Dryden A. J, Hughes J. T. etal. Homozygous prion protein genotype predisposes to sporadic CJD // Nature. - 1991. - Vol. 352. - P. 340-342.
  63. Pammer J., Suchy A., Rendi M. et al. Cellular prion protein expressed by bovine squamous epithelia of skin and upper gastrointestinal tract // Lancet. - 1999. - Vol. 359. - P. 1702- 1703.
  64. Pan K.-M., Baldwin M., Nguen J. et al. Conversion of a-helices into p-sheets features in the formation of the scrapie prion protein // Proc. Natl. Acad. Sci. USA. - 1993. - Vol. 90. - P. 10962-10966.
  65. Parchi P., Gambetti P. Human prion diseases // Curr. Opin. Neurol. - 1995. - Vol. 8. - P. 286-293.
  66. Parchi P., Zon W., Wang W. et al. Genetic influence on the structural variation of the abnormal prion protein // Proc. Natl. Acad. Sci. USA. - 2000. - Vol. 97. - P. 10168-10172.
  67. Pauly P., Harris D. A. Copper stimulates endocytosis of the prion protein // J. Biol. Chem. - 1998. - Vol. 73. - P. 33107- 33110.
  68. Petersen R. В., Parchi P., Richardson S. et al. Effect of the DI78N mutation and the codon 129 polymorphism of the prion protein // J. Biol. Chem. - 1996. - Vol. 271. - P. 12661- 12668.
  69. Prinz M., Montrasio F., Klein M. A. et al. Lymphnodal prion replication and neuroinvasion in mices devoid of FDC // Proc. Natl. Acad. Sci. USA. - 2002. - Vol. 99. - P. 919-924.
  70. Priola S. A. Prion portein diversity and disease in the TSE // Adv. Protein Chem. - 2001. - Vol. 57. - P. 1-27.
  71. Prusiner S. Prions // Proc. Natl. Acad. Sci. USA. - 1998. - Vol. 95. - P. 13363-13383.
  72. Prusiner S. B. Novel proteinaceous infectious particles cause scrapie // Science. - 1982. - Vol. 216. - P. 136-144.
  73. Prusiner S. B. The prion diseases // Brain Pathol. - 1998. - Vol. 8. - P. 499-513.
  74. Prusiner S. B. Molecular biology of prion diseases // Science. - 1991. - Vol. 252. - P. 1515-1522.
  75. Purdey M. Ecosystems supporting clusters of sporadic TSEs demonstrate excess of the radical-generating divalent cation manganese and deficiencies of antioxidant cofactors Cu, Se, Fe, Zn // Med. Hypothes. - 2000. - Vol. 54. - P. 278-306.
  76. Quaglio E., Chiesa R., Harris D. A. et al. Copper convert the cellular prion protein into a protease-resistant species that is distinct from the scrapie isoform // J. Biol. Chem. - 2001. - Vol. 276. - P. 11432.
  77. Race R., Meade-White K., Raines A. et al. Subclinical scrapie infection in a resistant species: persistence, replication, and adaptation of infectivity during four passages // J. Infect. Dis. - 2002. - Vol. 186. - Suppl. 2. - P. S166-S170.
  78. Rachidi W., Mange A., Senator A. et al. Prion infection impairs copper binding of culture cells // J. Biol. Chem. - 2003. - Vol. 278. - P. 14595-14598.
  79. Riek R., Hornemann S., Glockshuber R. et al. NMR characterization on the full-length recombinant murine prion // FEBS Lett. - 1997. - Vol. 413. - P. 282-288.
  80. Riesner D., Kelling K., Post K. et al. Disruption of prion rods generated 10 nm spherical particles having high a-helical content and lacking scrapie infectivity // J. Virol. - 1996. - Vol. 70. - P. 1714.
  81. Rossi G., Salmona M., Forloni G. et al. Therapeutic approaches to prion diseases // Clin. Lab. Med. - 2003. - Vol. 23. - P. 187-208.
  82. Rudd P. M., Wormald M. R., Wing D. R. et al. Prion glycoprotein: structure, dynamics and roles for the sugars // Biochemistry. - 2001. - Vol. 40. - P. 3761-3766.
  83. Saborio P., Permanne В., Soto С. Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding // Nature. - 2001. - Vol. 411. - P. 810-813.
  84. Safar J., Cohen F. E., Prusiner S. B. Quantitative traits of prion strains are unciphered in the conformation of the prion protein // Arch. Virol. - 2000. - Vol. 16. - Suppl. - P. 227-235.
  85. Shaked G. M., Shaked Y., Kariv-Inbal Z. et al. A protease-resistant prion protein isoform is present in urine of animals and humans affected with prion diseases // J. Biol. Chem. - 2001. - Vol. 276. - P. 34479-34482.
  86. Shaked Y., Rosenmann H., Talmor G., Gabizon R. Terminal trunkated PrP isoform is present in mature sperm // J. Biol. Chem. - 1999. - Vol. 274. - P. 2153-2158.
  87. Simonie Т., Duga S., Strumbo B. et al. cDNA cloning of turtle prion protein // FEBS Lett. - 2000. - Vol. 469. - P. 33-38.
  88. Soto C., Kascsak R. I., Saborio G. P. et al. Reversion of prion protein conformational changes by synthetic |3-sheet breaker peptide // Lancet. - 2000. - Vol. 355. - P. 192-197.
  89. Tobler L., Deboer Т., Fisher M. P. et al. Sleep and sleep regulation in normal and prion-deficient mice // J. Neurosci. - 1997. - Vol. 17. - P. 1869-1879.
  90. Van Rheede T. Smolenaars M. M., Madsen O. et al. Molecular evolution of the mammalian prion protein // Mol. Biol. Evol. - 2003. - Vol. 20. - P. 11-121.
  91. Viles J. H., Cohen F. E., Prusiner S. B. et al. Copper binding to the prion protein. Structural implications of four identical copper- active binding sites // Proc. Natl. Acad. Sci. USA. - 1999. - Vol. 96. - P. 2042-2047.
  92. Walmsley A. R., Zeng F., Hooper N. M. The N-terminal region of the prion protein ectodomain contain a lipid raft targeting determinant // J. Biol. Chem. - 2003. - Vol. 278. - P. 37241-37248.
  93. Wechselberger C., Wurm S., Pfarr W. et al. The physiological function of prion protein // Exp. Cell Res. - 2002. - Vol. 281. - P. 1-8.
  94. Will R. G., Ironside Y. W., Zeidler M. et al. A new variant of Creutzfeldt-Jakob disease in the UK // Lancet. - 1996. - Vol. 347. - P. 921-925.
  95. Wong B. S., Chen S. G., Colucci M. et al. Abberrant metal binding by prion protein in human prion disease // J. Neurochem. - 2001. - Vol. 78. - P. 1400-1408.
  96. Wu Q., Wang Y., Wang I. et al. The requirement of membrane lymphotoxin for the presence of dendritic cells in lymphoid tissue// J. Exp. Med. - 1999. - Vol. 190. - P. 629-638.
  97. Wuthrich K., Riek R. Three dimensional structures of prion protein // Adv. Protein Chem. - 2001. - Vol. 57. - P. 55-82.
  98. Zeidler M., Ironside I. W. The new variant of CID // Rev. Sci. Tech. - 2001. - Vol. 19. - P. 98-120.
  99. Zou W. Q., Capellari S., Parchi P. Identification of novel proteinase K-resistant C-terminal fragments of PrP in CJD // J. Biol. Chem. - 2003. - Vol. 278. - P. 40429-40436.

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