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Volume 20, Number 12—December 2014
Research

Variably Protease-Sensitive Prionopathy, a Unique Prion Variant with Inefficient Transmission Properties

Abigail B. Diack1, Diane Ritchie1, Alexander H. Peden, Deborah Brown, Aileen Boyle, Laura Morabito, David Maclennan, Paul Burgoyne, Casper Jansen, Richard S. Knight, Pedro Piccardo, James W. Ironside1, and Jean C. Manson1Comments to Author 
Author affiliations: The Roslin Institute, University of Edinburgh, Easter Bush, Scotland, UK (A.B. Diack, D. Brown, A, Boyle, L. Morabito, D. Maclennan, P. Burgoyne, J.C. Manson); School of Clinical Sciences, University of Edinburgh, Edinburgh, Scotland, UK (D.L. Ritchie, A.H. Peden, R.S. Knight, J.W. Ironside); Food and Drug Administration, Rockville, Maryland, USA (P. Piccardo); University Medical Centre Utrecht, Utrecht, the Netherlands (C. Jansen)

Main Article

Figure 3

Neuropathology in transgenic mice following inoculation with brain homogenate prepared from a postmortem sample from a person with VPSPr. Numerous PrP-labeled plaque-like deposits within the corpus callosum of HuVV (A) and HuMV (B) mice inoculated with brain homogenate from patient UK-VV. C) A single small PrP-labeled plaque in the stratum oriens of the hippocampus following experimental challenge with brain homogenate from patient NL-VV. D) PrP-labeled plaque-like-deposits in the corpus callosu

Figure 3. Neuropathology in transgenic mice following inoculation with brain homogenate prepared from a postmortem sample from a person with VPSPr. Numerous PrP-labeled plaque-like deposits within the corpus callosum of HuVV (A) and HuMV (B) mice inoculated with brain homogenate from patient UK-VV. C) A single small PrP-labeled plaque in the stratum oriens of the hippocampus following experimental challenge with brain homogenate from patient NL-VV. D) PrP-labeled plaque-like-deposits in the corpus callosum of a HuVV mouse inoculated with brain homogenate from patient UK-VV; inset: Thioflavin-S (Sigma, Gillingham, UK) staining of amyloid, viewed under ultraviolet light in a HuVV mouse challenged with brain homogenate from patient UK-VV. HuVV and HuMV, transgenic mice expressing human PrP gene sequence coding for the valine-homozygous and methionine/valine-heterozygous codon 129 genotypes, respectively; NL-VV and UK-VV, patients from the Netherlands and United Kingdom, respectively, who had VPSPr and the valine-homozygous codon 129 genotype; PrP, prion protein; VPSPr, variably protease-sensitive prionopathy. Scale bars indicate 25 μm.

Main Article

1These authors contributed equally to this article.

Page created: November 18, 2014
Page updated: November 18, 2014
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