NR AQLR
AU Kovacs,G.G.; Gasque,P.; Ströbel,T.; Lindeck-Pozza,E.; Strohschneider,M.; Ironside,J.W.; Budka,H.; Guentchev,M.
TI Complement activation in human prion disease
QU Neurobiology of Disease 2004 Feb; 15(1): 21-8
PT journal article
AB The central event in the neuropathological process of prion diseases (PrD) is the accumulation of abnormal prion protein accompanied by severe neuronal loss. Despite the infectious nature of these diseases, no prominent immune response has been detected yet. However, recent studies have shown that complement, a component of the innate immune system, is involved in the early pathogenesis of experimental prion infection. Here we demonstrate, in the diseased human brains, the presence of active compounds of the complement system, like C1q and C3b, in extracellular disease-associated prion protein deposits and the membrane attack complex in neurons. The neuronal localization of the membrane attack complex correlates well with the severity of disease-specific pathology and TUNEL labeling of neurons, irrespective of genotype or molecular phenotype of human prion diseases.
MH Adolescent; Adult; Aged; Aged, 80 and over; Antibodies/immunology; Antibody Specificity; Brain/*immunology/physiopathology; Complement/*immunology; Complement 1q/immunology; Complement 3b/immunology; Complement Membrane Attack Complex/*immunology; Female; Human; Immunity, Natural/immunology; Immunohistochemistry; Male; Middle Aged; Neurons/*immunology; PrPsc Proteins/immunology; Prion Diseases/*immunology; Support, Non-U.S. Gov't
AD Institute of Neurology, University of Vienna, Vienna, Austria.
SP englisch
PO USA