Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease

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

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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 (PrPSc) and the presumed cellular form (PrPC) 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 PrPC 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 PrPc.

Original languageEnglish
Pages (from-to)881-889
Number of pages9
JournalNeurobiology of Disease
Issue number5
Publication statusPublished - jan. 1 2001

ASJC Scopus subject areas

  • Neurology

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    Kovács, G. G., Kurucz, I., Budka, H., Ádori, C., Müller, F., Ács, P., Klöppel, S., Schätzl, H. M., Mayer, R. J., & László, L. (2001). Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease. Neurobiology of Disease, 8(5), 881-889.