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

Hafezparast, Majid, Brandner, Sebastian, Linehan, Jackie, Martin, Joanne E, Collinge, John and Fisher, Elizabeth M C (2004) Prion disease incubation time is not affected in mice heterozygous for a dynein mutation. Biochemical and Biophysical Research Communications, 326 (1). pp. 18-22. ISSN 0006-291X

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Abstract

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.

Item Type: Article
Additional Information: 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.
Schools and Departments: School of Life Sciences > Neuroscience
Depositing User: Majid Hafezparast
Date Deposited: 06 Feb 2012 19:55
Last Modified: 26 Mar 2012 12:54
URI: http://sro.sussex.ac.uk/id/eprint/23040
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