Projects per year
Abstract
Variably protease-sensitive prionopathy (VPSPr) can occur in persons of all codon 129 genotypes in the human prion protein gene (PRNP) and is characterized by a unique biochemical profile when compared with other human prion diseases. We investigated transmission properties of VPSPr by inoculating transgenic mice expressing human PRNP with brain tissue from 2 persons with the valine-homozygous (VV) and 1 with the heterozygous methionine/valine codon 129 genotype. No clinical signs or vacuolar pathology were observed in any inoculated mice. Small deposits of prion protein accumulated in the brains of inoculated mice after challenge with brain material from VV VPSPr patients. Some of these deposits resembled microplaques that occur in the brains of VPSPr patients. Comparison of these transmission properties with those of sporadic Creutzfeldt-Jakob disease in the same lines of mice indicated that VPSPr has distinct biological properties. Moreover, we established that VPSPr has limited potential for human-to-human transmission.
Original language | English |
---|---|
Pages (from-to) | 1969-1979 |
Number of pages | 11 |
Journal | Emerging Infectious Diseases |
Volume | 20 |
Issue number | 12 |
DOIs | |
Publication status | Published - 1 Jan 2014 |
Fingerprint
Dive into the research topics of 'Variably protease-sensitive prionopathy, a unique prion variant with inefficient transmission properties'. Together they form a unique fingerprint.Projects
- 1 Active
Profiles
-
Abigail Diack
- Royal (Dick) School of Veterinary Studies - Roslin Research Fellow
Person: Academic: Research Active
-
Richard Knight
- Centre for Clinical Brain Sciences
- Edinburgh Neuroscience
- Deanery of Clinical Sciences - Professorial Fellow
Person: Academic: Research Active
-
Jean Manson
- Deanery of Clinical Sciences - Professorial Fellow
- Centre for Clinical Brain Sciences
Person: Academic: Research Active