Abstract
Prion diseases belong to the group of neurodegenerative diseases that are very rare in general population and are registered as sporadic, infectious and inherited forms. These diseases are caused by contagious prion protein that appears as the result of conformational changes of normal host protein on posttranslational stage. There is phenotypical heterogenesis of prion diseases is the result of interreaction of several factors. The most important of them seems to be the expression of the protein PrPres and polymorphism in codon 129. Lifetime diagnosis of prion diseases is insufficient and there is no reliable options for treatment of these disease as well as for specific prophylaxis.
-
1.
Palmer M.S., Collinge J. Prion diseases: an introduction. In: Collinge J., Palmer M.S., editors. Prion Diseases. Oxford: Oxford University Press; 1997.
-
2.
Prusiner S.B. Human prion diseases and neurodegeneration: prions, prions, prions. Berlin. Heidelberg; 1998. p.1-17.
-
3.
Зуев В.А. Медленные вирусные инфекции человека и животных. М.: Медицина; 1988.
-
4.
Korczyn A.D. Human prion diseases. Proceedings of 49th Annual meeting of American Academy of Neurology; 1997 Apr 12-19, Boston, MA. p.1-19.
-
5.
Chazot G., Brousolle E., Lapras CI., Blatter T., Aguzzi A., Kopp N. New variant of Creutzfeld-Jacob Disease in a 26-year-old French man. Lancet 1996; 347:1181.
-
6.
Will R.G., Ironside J.W., Zeidler M., et al. A new variant of Creutzfeldt-Jacob Disease in the UK. Lancet 1996; 347:921-5.
-
7.
Bruce M.E., Will R.G., Ironside J.W., McConnell J., et al. Transmissions to mice indicate that "new variant" Creutzfeldt-Jacob disease is caused by the BSE agent. Nature 1997; 389:498-501.
-
8.
Hill A.F., Zeidler M., Ironside J., Collinge J. Diagnosis of new variant Creutzfeldt-Jacob disease by tonsil biopsy. Lancet 1996; 347:921-5.
-
9.
Brandner S., Iseman S., Raeber A., et al. Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 1996; 379:339-40.
-
10.
Prusiner S.B. Prions causing neurodegenerative diseases of humans and animals. In: Jolles G., Stutzmann J.M., editors. Neurodegenerative diseases. Acad Press; 1996. p.23-80.
-
11.
Зуев В.А., Завалишин И.А., Ройхель В.М. Прионные болезни человека и животных. Руководство для врачей. М.: Медицина; 1999.
-
12.
Hay B., Prusiner S.B., Lingappa V.R. Biochemistry 1987; 26:8110-4.
-
13.
Ironside I.W. Human prion diseases. J Neural Transm 1996; 47:231-46.
-
14.
Prusiner S.B. Human prion diseases and neurodegeneration. Cur Topics in Microb Immunol 1996; 207:1-17.
-
15.
Gambetti P. Human prion diseases. Proceedings of the 49th Annual meeting of American Academy of Neurology; 1997 Apr 12-19; Boston, MA. p.43-62.
-
16.
Alperovitch A. Epidemiology of Creutzfeldt-Jacob disease – past and present uncertainties. Eur J Neurol 1996; 3:500-6.
-
17.
Bell J., Ironside J. Neuropathology of spongiform encephalopathies in humans. Br Med Bull 1993; 49:738-77.
-
18.
Budka H., Aguzzi A., Brown P. Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (Prion diseases). Brain Pathol 1995; 5:459-66.
-
19.
Report of a WHO Consultation on Clinical and Neuropathological Characteristics of the New Variant of Creutzfeldt-Jacob disease and other Human and Animal Transmissible Spongiform encephalopathies; 1996, 1997, 1998, Geneva.
-
20.
Matsuda H. Chicken monoclonal antibodies with specificity for the N-terminal of human prion protein. FIMS Immunol Med Microbiol 1999; 23:189-94.
-
21.
Hsich G., Kenney K., Gibbs C.J., Lee K.H., Harrington M.G. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopaties. N Engl J Med 1996; 335:924-30.
-
22.
Завалишин И.А., Адарчева Л.С., Ройхель В.М., Фомина Г.И., Кондакова Л.И., Соболев С.Г., Погодина В.В. Синдром Герстманна-Штреусслера: новые возможности диагностики. Журн невропатол и психиатр 1995; 1:58-63.
-
23.
Medori R., Tritchler H-J., LeBlanc A., et al. Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med 1992; 326:444-9.
-
24.
Gambetti P., Parchi P. Insomnia in prion diseases: sporadic and familial. N Engl J Med 1999; 340:1675-7.
-
25.
Gajdusek D.C. Infectious amyloids: subacute spongiform encephalopathies as transmissible cerebral amyloidoses. In: Fields B.N., et al., editors. Fields Virology. Philadelphia: Lippincott Raven Publishers; 1996. p.2851-900.
-
26.
Collinge J., Palmer M.S., Sidle K.C., et al. Unaltered susceptibility to BSE in transgenic mice expressing human prion protein. Nature 1995; 378:779-83.
-
27.
Cousens S.N., et al. Predicting the CJD epidemic in humans. Nature 1997; 385:197-8.