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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

Table 2

Results of intracerebral inoculation of brain tissue homogenates from 3 patients with variably protease-sensitive prionopathy into 3 lines of human transgenic mice*

Brain inoculum source, mouse line† No. mice positive/no. total
 Mean no. PrP plaque–like deposits (range)¶
Clinical signs of prion disease‡ Vacuolar degeneration‡ PrP deposition§
UK-VV
HuMM 0/15 0/15 1/15 0#
HuMV 4/15 0/15 2/14 5 (2–8)
HuVV
 0/14
 0/14
 5/14
 10 (1–17)
UK-MV
HuMM 1/15 0/15 0/15 0
HuMV 1/15 0/15 0/15 0
HuVV
 0/15
 0/15
 0/15
 0
NL-VV
HuMM 0/15 0/15 0/15 0
HuMV 0/15 0/15 3/15 8 (1–15)
HuVV 0/14 0/14 7/14 3 (2–4)

*HuMM, HuMV, and HuVV, transgenic mice expressing the different forms of the human PrP gene (i.e., those homozygous for methionine [MM] or valine [VV] or heterozygous for methionine and valine [MV]); PrP, prion protein.
†Brain inoculum was prepared from postmortem samples from persons with variably protease-sensitive prionopathy. NL-VV, patient from the Netherlands who had the PrP codon 129VV genotype; UK-MV, patient from the United Kingdom who had the PrP codon 129MV genotype; UK-VV, patient from the United Kingdom who had the PrP codon 129VV genotype.
‡In mice with a positive score for clinical signs of a prion disease and a negative score for vacuolar pathology, the neuropathologic assessment was considered definitive.
§A positive score for PrP pathology was given to mice showing PrP deposition in the brain with at least 1 of the 4 PrP antibodies used in the immunohistochemical analysis.
¶The number of plaque-like deposits was counted per mouse, and results are given as mean (range) for each genotype.
#Mouse showed evidence of PrP deposition in a tumor.

Main Article

1These authors contributed equally to this article.

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