Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease.

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).

Statistics

0200400600'04'05'06'07'08'09'10'11'12'13'14'15'16'17
Citations per Year

1,197 Citations

Semantic Scholar estimates that this publication has 1,197 citations based on the available data.

See our FAQ for additional information.

Cite this paper

@article{Kovacs2001ProminentSR, title={Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease.}, author={Gabor G Kovacs and Istv{\'a}n Kurucz and Herbert Budka and Csaba {\'A}dori and F. M{\"{u}ller and Peter G Acs and Stefan Kl{\"{o}ppel and Hermann M. Sch{\"a}tzl and Robert J. Mayer and Lajos L{\'a}szl{\'o}}, journal={Neurobiology of disease}, year={2001}, volume={8 5}, pages={881-9} }