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InFo Neurologie & Psychiatrie

, Volume 15, Issue 6, pp 42–49 | Cite as

Prionerkrankungen — welche Rolle spielen sie heute?

Creutzfeldt-Jakob-Erkrankung
  • Claudia PontoEmail author
  • Inga Zerr
zertifizierte fortbildung

Zusammenfassung

Das Konzept der Protein-Fehlfaltung als Ursache einer Neurodegeneration wie der Creutzfeldt-Jakob-Erkrankung (CJK) wurde bei der Gruppe der Prionerkrankungen erstmalig beschrieben. Obwohl diese als vergleichsweise selten und exotisch betrachtet werden, hatten sie letztlich einen großen Stellenwert für das Verständnis pathogenetischer Mechanismen bei anderen neurodegenerativen Erkrankungen.

Literatur

  1. 1.
    Creutzfeldt HG. Über eine eigenartige herdförmige Erkrankung des Zentralnervensystems (vorläufige Mitteilung). Z ges Neurol Psychiat 1920; 57: 1–18CrossRefGoogle Scholar
  2. 2.
    Jakob A. Über eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswerten anatomischen Befunden (spastische Pseudosklerose-Encephalomyelopathie mit disseminierten Degenerationsherden). Dtsch Z Nervenheilk 1921; 70: 132–146Google Scholar
  3. 3.
    Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982; 216 (4542): 136–144PubMedCrossRefGoogle Scholar
  4. 4.
    Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE. Purification and structural studies of a major scrapie prion protein. Cell 1984; 38 (1): 127–134PubMedCrossRefGoogle Scholar
  5. 5.
    Cuillé J, Chelle PL. Experimental transmission of trembling to the goat. Comptes Rendus des Seances Acad Sci 1939; 208: 1058–1160Google Scholar
  6. 6.
    Alperovitch A, Zerr I, Pocchiari M, Mitrova E, de Pedro Cuesta J, Hegyi I, Collins S, Kretschmar H, van Duijn C, Will RG. Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 1999; 353 (9165): 1673–1674PubMedCrossRefGoogle Scholar
  7. 7.
    Palmer MS, Dryden AJ, Hughes JT, Collinge J. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 1991; 352 (6333): 340–342PubMedCrossRefGoogle Scholar
  8. 8.
    Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, Farlow M, Dickson DW, Sima AA, Trojanowski JQ, Petersen RB, Gambetti P. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39 (6): 767–778PubMedCrossRefGoogle Scholar
  9. 9.
    Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, Breithaupt M, Varges D, Meissner B, Ladogana A, Schuur M, Haik S, Collins SJ, Jansen GH, Stokin GB, Pimentel J, Hewer E, Collie D, Smith P, Roberts H, Brandel JP, van Duijn C, Pocchiari M, Begue C, Cras P, Will RG, Sanchez-Juan P. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009; 132 (Pt 10): 2659–2668PubMedCrossRefGoogle Scholar
  10. 10.
    Zerr I, Bodemer M, Gefeller O, Otto M, Poser S, Wiltfang J, Windl O, Kretschmar HA, Weber T. Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease. Ann Neurol 1998; 43 (1): 32–40PubMedCrossRefGoogle Scholar
  11. 11.
    Stoeck K, Sanchez-Juan P, Gawinecka J, Green A, Ladogana A, Pocchiari M, Sanchez-Valle R, Mitrova E, Sklaviadis T, Kulczycki J, Slivarichova D, Saiz A, Calero M, Knight R, Aguzzi A, Laplanche JL, Peoc’h K, Schelzke G, Karch A, van Duijn CM, Zerr I. Cerebrospinal fluid biomarker supported diagnosis of Creutzfeldt-Jakob disease and rapid dementias: a longitudinal multicentre study over 10 years. Brain 2012; 135 (Pt 10): 3051–3061PubMedCrossRefGoogle Scholar
  12. 12.
    Gawinecka J, Dieks J, Asif AR, Carimalo J, Heinemann U, Streich JH, Diahzi H, Schulz-Schaeffer W, Zerr I. Codon 129 polymorphism specific cerebrospinal fluid proteome pattern in sporadic Creutzfeldt-Jakob disease and the implication of glycolytic enzymes in prion-induced pathology. J Proteome Res 2010; 9 (11): 5646–5657PubMedCrossRefGoogle Scholar
  13. 13.
    Zafar S, von Ahsen N, Oellerich M, Zerr I, Schulz-Schaeffer WJ, Armstrong VW, Asif AR. Proteomics approach to identify the interacting partners of cellular prion protein and characterization of Rab7a interaction in neuronal cells. J Proteome Res 2011; 10 (7): 3123–3135PubMedCrossRefGoogle Scholar
  14. 14.
    Atarashi R, Satoh K, Sano K, Fuse T, Yamaguchi N, Ishibashi D, Matsubara T, Nakagaki T, Yamanaka H, Shirabe S, Yamada M, Mizusawa H, Kitamoto T, Klug G, McGlade A, Collins SJ, Nishida N. Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion. Nat Med 2011; 17 (2): 175–178PubMedCrossRefGoogle Scholar
  15. 15.
    Fincke F. Doxycyclin bei der sporadischen Creutzfeldt-Jakob-Krankheit. Dissertation, Universitätsmedizin Göttingen 2010Google Scholar
  16. 16.
    Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347 (9006): 921–925PubMedCrossRefGoogle Scholar
  17. 17.
    Hewitt PE, Llewelyn CA, Mackenzie J, Will RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study. Vox Sang 2006; 91 (3): 221–230PubMedCrossRefGoogle Scholar
  18. 18.
    Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004; 364 (9433): 527–529PubMedCrossRefGoogle Scholar
  19. 19.
    Bishop MT, Diack AB, Ritchie DL, Ironside JW, Will RG, Manson JC. Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease. Brain 2013; 136 (Pt 4): 1139–1145PubMedCrossRefGoogle Scholar
  20. 20.
    Jucker M, Walker LC. Pathogenetic protein seeding in Alzheimer’s disease and other neurodegenerative disorders. Ann Neurol. 2011; 70 (4): 532–540PubMedCrossRefGoogle Scholar
  21. 21.
    Eisele YS, Obermüller U, Heilbronner G, Baumann F, Kaeser SA, Wolburg H, Walker LC, Staufenbiel M, Heikenwalder M, Jucker M. Peripherally applied Abeta-containing inoculates induce cerebral beta-amyloidosis. Science 2010; 330 (6006): 980–982PubMedCrossRefGoogle Scholar
  22. 22.
    Braak H, Del Tredici K, Rüb U, de Vos RA, Jansen Steur EN, Braak E. Staging of brain pathology related to sporadic Parkinson’s disease. Neurobiol Aging. 2003; 24 (2): 197–211PubMedCrossRefGoogle Scholar
  23. 23.
    Li JY, Englund E, Holton JL, et al. Lewy bodies in grafted neurons in subjects with Parkinson’s disease suggest host-to-graft disease propagation. Nat Med. 2008; 14 (5): 501–503PubMedCrossRefGoogle Scholar
  24. 24.
    Kordower JH, Chu Y, Hauser RA, Freeman TB, Olanow CW. Lewy body-like pathology in long-term embryonic nigral transplants in Parkinson’s disease. Nat Med. 2008; 14 (5): 504–506PubMedCrossRefGoogle Scholar
  25. 25.

Copyright information

© Urban & Vogel 2013

Authors and Affiliations

  1. 1.Prionforschungsgruppe Neurologische Klinik, UniversitätsmedizinGöttingenDeutschland

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