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

Hafezparast, M; Brandner, S; Linehan, J; Martin, JE; Collinge, J; Fisher, EMC; (2005) Prion disease incubation time is not affected in mice heterozygous for a dynein mutation. BIOCHEM BIOPH RES CO , 326 (1) 18 - 22. 10.1016/j.bbrc.2004.10.206.

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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. (C) 2004 Elsevier Inc. All rights reserved.

Type: Article
Title: Prion disease incubation time is not affected in mice heterozygous for a dynein mutation
DOI: 10.1016/j.bbrc.2004.10.206
Keywords: Prion disease, PrP, Loa, cytoplasmic dynein, retrograde transport, LIGHT-CHAIN, RETROGRADE TRANSPORT, AXONAL-TRANSPORT, PROTEIN, CELLS, NEUROINVASION, DEGENERATION, PATHOGENESIS, SCRAPIE, SPREAD
UCL classification: UCL > School of Life and Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Neurology
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Neurology > Neurodegenerative Diseases
URI: http://discovery.ucl.ac.uk/id/eprint/145881
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