NR ARRJ
AU Liberski,P.P.; Sikorska,B.; Bratosiewicz-Wasik,J.; Gajdusek,D.C.; Brown,P.
TI Neuronal cell death in transmissible spongiform encephalopathies (prion diseases) revisited: from apoptosis to autophagy.
QU International Journal of Biochemistry and Cell Biology 2004 Dec; 36(12): 2473-90
PT journal article; review; review, tutorial
AB Neuronal autophagy, like apoptosis, is one of the mechanisms of the programmed cell death (PCD). In this review, we summarize the presence of autophagic vacuoles in experimentally induced scrapie, Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker (GSS) syndrome. Initially, a part of the neuronal cytoplasm was sequestrated by concentric arrays of double membranes; the enclosed cytoplasm appeared relatively normal except that its density was often increased. Next, electron density of the central area dramatically increased. The membranes then proliferated within the cytoplasm in a labyrinth-like manner and the area sequestrated by these membranes enlarged into a more complex structure consisting of vacuoles, electron-dense areas and areas of normally-looking cytoplasm connected by convoluted membranes. Of note, autophagic vacuoles form not only in neuronal perikarya but also in neurites and synapses. Finally, a large area of the cytoplasm was transformed into a collection of autophagic vacuoles of different sizes. On a basis of ultrastructural studies, we suggest that autophagy plays a major role in transmissible spongiform encephalopathies (TSEs) and may even participate in a formation of spongiform change.
ZR 142
MH Animals; Apoptosis/*physiology; Autophagocytosis/*physiology; Cell Death/physiology; Humans; Neurons/pathology/*physiology; Prion Diseases/pathology/*physiopathology; Prions/physiology; Research Support, Non-U.S. Gov't; Vacuoles/physiology
AD Pawel P. Liberski (ppliber@csk.am.lodz.pl), Beata Sikorska, D. Carleton Gajdusek, Department of Molecular Pathology and Neuropathology, Medical University Lodz, Czechoslowacka Street 8/10; pl 92-216 Lodz, Poland; Jolanta Bratosiewicz-Wasik, D. Carleton Gajdusek, Department of Virology, Medical University of Silesia in Katowice, Poland; Paul Brown, Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, USA
SP englisch
PO England