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

First Author  Hafezparast M Year  2005
Journal  Biochem Biophys Res Commun Volume  326
Issue  1 Pages  18-22
PubMed ID  15567146 Mgi Jnum  J:94178
Mgi Id  MGI:3511417 Doi  10.1016/j.bbrc.2004.10.206
Citation  Hafezparast M, et al. (2004) Prion disease incubation time is not affected in mice heterozygous for a dynein mutation. Biochem Biophys Res Commun 326(1):18-22
abstractText  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.
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