title: Size frequency distributions of the florid prion protein aggregates in variant Creutzfeldt-Jakob disease follow a power-law function.
abstract: The objective was to test the hypothesis that the size frequency distributions of the prion protein (PrP) plaques in cases of variant Creutzfeldt-Jakob disease (vCJD) follow a power-law function. The design was a retrospective neuropathological study. The patients were 11 cases of clinically and neuropathologically verified vCJD. Size distributions of the diffuse and florid-type plaques were measured in several areas of the cerebral cortex and hippocampus from each case and a power-law function fitted to each distribution. The size distributions of the florid and diffuse plaques were fitted successfully by a powerlaw function in 100% and 42% of brain areas investigated respectively. Processes of aggregation/disaggregation may be more important than surface diffusion in the pathogenesis of the florid plaques. By contrast, surface diffusion may be a more significant factor in the development of the diffuse plaques.
authors.affiliation: Vision Sciences, Aston University, Birmingham B4 7ET, and National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK. R.A.Armstrong@aston.ac.uk
authors.forename: R A
authors.initials: RA
authors.lastname: Armstrong
authors.forename: N J
authors.initials: NJ
authors.lastname: Cairns
authors.forename: J W
authors.initials: JW
authors.lastname: Ironside
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011328:Prions / Q000378:metabolism*; D012189:Retrospective Studies
chemical_list: D011328:Prions