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Prion disease incubation time is not affected in mice heterozygous for a dynein mutation.
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posted on 2023-06-08, 00:36 authored by Majid HafezparastMajid Hafezparast, Sebastian Brandner, Jackie Linehan, Joanne E Martin, John Collinge, Elizabeth M C FisherA mechanism for transmission of the infectious prions from the peripheral nerve ends to the central nervous system is thought to involve neuronal anterograde and retrograde transport systems. Cytoplasmic dynein is the major retrograde transport molecular motor whose function is impaired in the Legs at odd angles (Loa) mouse due to a point mutation in the cytoplasmic dynein heavy chain subunit. Loa is a dominant trait which causes neurodegeneration and progressive motor function deficit in the heterozygotes. To investigate the role of cytoplasmic dynein in the transmission of prions within neurons, we inoculated heterozygous Loa and wild type littermates with mouse-adapted scrapie prions intracerebrally and intraperitonially, and determined the incubation period to onset of clinical prion disease. Our data indicate that the dynein mutation in the heterozygous state does not affect prion disease incubation time or its neuropathology in Loa mice.
History
Publication status
- Published
Journal
Biochemical and Biophysical Research CommunicationsISSN
0006-291XExternal DOI
Issue
1Volume
326Page range
18-22Pages
5.0Department affiliated with
- Neuroscience Publications
Notes
MH carried out the analysis and co-authored the paper in collaboration with J. Collinge, E.M.C. Fisher, and S. Brandner. Here the role of retrograde axonal transport in the entry of prion agent into CNS was studied. This paper illustrated the use of Loa mouse as a tool for such studies.Full text available
- No
Peer reviewed?
- Yes
Legacy Posted Date
2012-02-06Usage metrics
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