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Prion disease incubation time is not affected in mice heterozygous for a dynein mutation.

journal contribution
posted on 2023-06-08, 00:36 authored by Majid HafezparastMajid Hafezparast, Sebastian Brandner, Jackie Linehan, Joanne E Martin, John Collinge, Elizabeth M C Fisher
A 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 Communications

ISSN

0006-291X

Issue

1

Volume

326

Page range

18-22

Pages

5.0

Department 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

2012-02-06

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