Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease

Gábor G. Kovács, István Kurucz, Herbert Budka, C. Ádori, Ferenc Müller, Péter Ács, Stefan Klöppel, Hermann M. Schätzl, R. John Mayer, L. László

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

To examine the role of stress-related 70-kDa heat shock proteins (Hsp-s) in Creutzfeldt - Jakob disease (CJD), we performed immunocytochemistry to detect Hsp-72 and Hsp-73, together with the abnormal (PrP Sc) and the presumed cellular form (PrP C) of the prion protein, and TUNEL method to measure cellular vulnerability in different brain regions in CJD and control cases. While Hsp-73 showed uniform distribution in all the examined samples, an increase in the number of Purkinje cells with prominent accumulation of Hsp-72 in the CJD group was observed. These neurons also showed intense PrP C staining, but TUNEL-positive nuclei were only detected in the granular (Hsp-72-negative) cell layer. Fewer cells of the inferior olivary nucleus were immunoreactive for Hsp-72 in CJD than in control cases, and regions showing severe spongiform change and gliosis exhibited fewer Hsp-72-immunoreactive neurons. Our results indicate that accumulation of the inducible Hsp-72 in certain cell types may be part of a cytoprotective mechanism, which includes preservation of proteins like PrP c.

Original languageEnglish
Pages (from-to)881-889
Number of pages9
JournalNeurobiology of Disease
Volume8
Issue number5
DOIs
Publication statusPublished - 2001

Fingerprint

Creutzfeldt-Jakob Syndrome
Purkinje Cells
In Situ Nick-End Labeling
Olivary Nucleus
Neurons
HSP70 Heat-Shock Proteins
Gliosis
Immunohistochemistry
Staining and Labeling
Brain
Prion Proteins

Keywords

  • Creutzfeldt-Jakob disease
  • Cytoprotection
  • Heat shock proteins
  • Prion
  • Purkinje cell

ASJC Scopus subject areas

  • Neurology

Cite this

Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease. / Kovács, Gábor G.; Kurucz, István; Budka, Herbert; Ádori, C.; Müller, Ferenc; Ács, Péter; Klöppel, Stefan; Schätzl, Hermann M.; Mayer, R. John; László, L.

In: Neurobiology of Disease, Vol. 8, No. 5, 2001, p. 881-889.

Research output: Contribution to journalArticle

Kovács, GG, Kurucz, I, Budka, H, Ádori, C, Müller, F, Ács, P, Klöppel, S, Schätzl, HM, Mayer, RJ & László, L 2001, 'Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease', Neurobiology of Disease, vol. 8, no. 5, pp. 881-889. https://doi.org/10.1006/nbdi.2001.0418
Kovács, Gábor G. ; Kurucz, István ; Budka, Herbert ; Ádori, C. ; Müller, Ferenc ; Ács, Péter ; Klöppel, Stefan ; Schätzl, Hermann M. ; Mayer, R. John ; László, L. / Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease. In: Neurobiology of Disease. 2001 ; Vol. 8, No. 5. pp. 881-889.
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