Prominent Stress Response of Purkinje Cells in Creutzfeldt–Jakob Disease

Neurobiology of Disease

Csaba Ádori¹  Lajos László¹  Herbert Budka²  R.John Mayer²  Ferenc Müller³  Hermann M. Schätzl⁴  István Kurucz⁴  Péter Ács⁵  Stefan Klöppel⁶  Gábor G. Kovács⁶ 

[1] Department of General Zoology, Eötvös University of Sciences, H-1088, Budapest, Hungary;Department of Neurobiochemistry, Institute of Drug Research Ltd. Budapest, Hungary;Genecenter Munich, Max von Pettenkofer Institute, University of Munich, Munich, Germany;Institute of Neurology, University of Vienna, Austrian Reference Center for Human Prion Diseases, Vienna, Austria;Laboratory of Intracellular Proteolysis, School of Biomedical Sciences, University of Nottingham Medical School, Queen's Medical Centre, Nottingham, United Kingdom;Department of Neurology, Semmelweis University of Medicine, Budapest, Hungary;

关键词: prion;    Creutzfeldt–Jakob disease;    Purkinje cell;    heat shock proteins;    cytoprotection;   

DOI  :  

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown