NR AGTI
AU Kovacs,G.G.; Kurucz,I.; Budka,H.; Adori,C.; Müller,F.; Acs,P.; Klöppel,S.; Schätzl,H.M.; Mayer,R.J.; Laszlo,L.
TI Prominent stress response of Purkinje cells in Creutzfeldt-Jakob disease
QU Neurobiology of Disease 2001 Oct; 8(5): 881-9
PT journal article
AB 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.
MH Adult; Aged; Apoptosis; Astrocytes/metabolism; Carrier Proteins/*metabolism; Creutzfeldt-Jakob Syndrome/*metabolism/pathology; Female; Gliosis/metabolism; Heat-Shock Proteins/*metabolism; Human; In Situ Nick-End Labeling; Male; Medulla Oblongata/metabolism; Middle Age; Nerve Tissue Proteins/*metabolism; Olivary Nucleus/metabolism; Phosphoprotein Phosphatase/chemistry/*metabolism; Protein Conformation; Protein Folding; Purkinje Cells/*physiology; Stress/*physiopathology; Support, Non-U.S. Gov't
AD Department of Neurology, Semmelweis University of Medicine, Budapest, Hungary.
SP englisch
PO USA