Size frequency distribution of prion protein (PrP) aggregates in variant Creutzfeldt-Jakob disease (vCJD)

Richard A. Armstrong*, Nigel J. Cairns, J.W. Ironside, Peter L. Lantos

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

The frequency distribution of aggregate size of the diffuse and florid-type prion protein (PrP) plaques was studied in various brain regions in cases of variant Creutzfeldt-Jakob disease (vCJD). The size distributions were unimodal and positively skewed and resembled those of β-amyloid (Aβ) deposits in Alzheimer's disease (AD) and Down's syndrome (DS). The frequency distributions of the PrP aggregates were log-normal in shape, but there were deviations from the expected number of plaques in specific size classes. More diffuse plaques were observed in the modal size class and fewer in the larger size classes than expected and more florid plaques were present in the larger size classes compared with the log-normal model. It was concluded that the growth of the PrP aggregates in vCJD does not strictly follow a log-normal model, diffuse plaques growing to within a more restricted size range and florid plaques to larger sizes than predicted. © Springer-Verlag 2005.

Original languageEnglish
Pages (from-to)1565-1573
Number of pages9
JournalJournal of Neural Transmission
Volume112
Issue number11
DOIs
Publication statusPublished - Nov 2005

Fingerprint

Creutzfeldt-Jakob Syndrome
Alzheimer Disease
Amyloid Plaques
Down Syndrome
Brain
Growth
Protein Aggregates
Prion Proteins

Keywords

  • variant Creutzfeldt-Jakob disease
  • prion protein aggregate
  • size distribution
  • log-normal distribution
  • florid plaque
  • diffuse plaque
  • Alzheimer's disease
  • beta-amyloid

Cite this

Armstrong, Richard A. ; Cairns, Nigel J. ; Ironside, J.W. ; Lantos, Peter L. / Size frequency distribution of prion protein (PrP) aggregates in variant Creutzfeldt-Jakob disease (vCJD). In: Journal of Neural Transmission. 2005 ; Vol. 112, No. 11. pp. 1565-1573.
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Size frequency distribution of prion protein (PrP) aggregates in variant Creutzfeldt-Jakob disease (vCJD). / Armstrong, Richard A.; Cairns, Nigel J.; Ironside, J.W.; Lantos, Peter L.

In: Journal of Neural Transmission, Vol. 112, No. 11, 11.2005, p. 1565-1573.

Research output: Contribution to journalArticle

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