Distribution of apoptosis-related proteins in sporadic Creutzfeldt–Jakob disease

• Published in: Brain research Vol. 1323; pp. 192 - 199
• Main Authors: Kovacs, Gabor G, Budka, Herbert
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 06.04.2010; Elsevier
• Subjects: Aged; Apoptosis; Bax, caspase; bcl-2-Associated X Protein - metabolism; Biological and medical sciences; Brain - metabolism; Brain - pathology; Caspase 3 - metabolism; Caspase 9 - metabolism; Cell Count; Chi-Square Distribution; Creutzfeldt-Jakob Syndrome - metabolism; Creutzfeldt-Jakob Syndrome - pathology; Creutzfeldt–Jakob disease; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Humans; Immunohistochemistry; Male; Medical sciences; Middle Aged; Neurology; Neurons - metabolism; Neurons - pathology; NF-kappa B - metabolism; PARP; Prion; Proto-Oncogene Proteins c-jun - metabolism; Bax, caspase; PARP; Prion; Creutzfeldt–Jakob disease; Apoptosis; Human; Prion disease; Nervous system diseases; Sporadic; Enzyme; Cysteine endopeptidases; Creutzfeldt-Jakob disease; Caspase; Protein; Cerebral disorder; Infection; Peptidases; Cell death; Central nervous system disease; Distribution; Bax; Hydrolases; Degenerative disease
• ISSN: 0006-8993; 1872-6240
• DOI: 10.1016/j.brainres.2010.01.089

Abstract Apoptosis is one mode of programmed cell death in sporadic Creutzfeldt–Jakob disease (sCJD). Distribution of affected brain regions varies between subtypes of sCJD. To compare apoptosis-related proteins representing different stages of cell death, we evaluated 30 anatomical regions for PARP, NF-κB, C-Jun, caspase 3 and 9, and BAX immunoreactivity in 12 sCJD cases of MM-1, MV-1, VV-2, and MV-2 subtypes, and 4 control brains. Chi-square test was used to compare the regional presence of immunoreactivities in sCJD cases and controls. The total number of subregions showing neuronal immunoreactivity for any apoptosis-related protein, in particular caspase-3, BAX, and PARP, was significantly higher in sCJD than controls, differed between subtypes of sCJD, and inversely correlated with duration of illness. Thus, sCJD subtypes are both qualitatively and regionally distinct in the predominance of apoptosis-related proteins, confirming regional vulnerabilities of sCJD subtypes that potentially reflect distinct responses to pathogenetic events.