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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 4

Neuropathology in transgenic mice following inoculation with brain homogenate prepared from a postmortem sample from a person with VPSPr. A–C) Immunohistochemistry for the PrP, showing small granular and microplaque-like deposits within the CA3 region of the hippocampus of a HuVV mouse challenged with VPSPr inoculum prepared from patient UK-VV. Differential staining was observed in this mouse by using the following PrP antibodies: Purified (3F4) (Cambridge Bioscience, Cambridge, UK) (A); Prion P

Figure 4. Neuropathology in transgenic mice following inoculation with brain homogenate prepared from a postmortem sample from a person with VPSPr. A–C) Immunohistochemistry for the PrP, showing small granular and microplaque-like deposits within the CA3 region of the hippocampus of a HuVV mouse challenged with VPSPr inoculum prepared from patient UK-VV. Differential staining was observed in this mouse by using the following PrP antibodies: Purified (3F4) (Cambridge Bioscience, Cambridge, UK) (A); Prion Protein Monoclonal Antibody (12F10) (Bioquote Ltd, York, UK) (B); and mAb 6H4 (Prionics AG, Schlieren, Switzerland) (C). D) Molecular layer of the cerebellum of a person with VPSPr, showing microplaque deposits stained with monoclonal antibody 3F4. HuVV, transgenic mouse expressing human PrP gene sequence coding for the valine-homozygous codon 129 genotype; PrP, prion protein; UK-VV, patient from the United Kingdom who had VPSPr and the valine-homozygous codon 129 genotype; VPSPr, variably protease-sensitive prionopathy. Scale bars indicate 25 μm.

Main Article

1These authors contributed equally to this article.

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