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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 3

Results of intracerebral inoculation of brain tissue homogenates from 4 patients with different subtypes of the sporadic form of Creutzfeldt-Jakob disease into 3 lines of human transgenic mice*

Brain inoculum source, mouse line† No. mice positive/no. total
Clinical signs of prion disease Vacuolar degeneration PrP deposition
MM1
HuMM 10/13 13/13 13/13
HuMV 9/14 14/14 14/14
HuVV
 8/16
 13/16
 14/16
MM2
HuMM 0/16 0/16 0/15
HuMV 0/18 0/18 2/18
HuVV
 0/17
 0/17
 3/17
VV1
HuMM 0/16 0/16 2/16
HuMV 2/14 9/14 1/14
HuVV
 2/14
 7/14
 7/14
VV2
HuMM 4/18 6/18 15/17
HuMV 1/15 5/15 12/14
HuVV 13/16 16/16 15/15

*Data adapted from Bishop et al. (3). 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 who had the MM1, MM2, VV1, or VV2 subtype of sporadic Creutzfeldt-Jakob disease.

Main Article

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Main Article

1These authors contributed equally to this article.

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Page updated: November 18, 2014
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