Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent.

Abstract:

:To study the effect of cell type-restricted hamster PrP expression on susceptibility to the hamster scrapie agent, we generated transgenic mice using a 1 kb hamster cDNA clone containing the 0.76 kb HPrP open reading frame under control of the neuron-specific enolase promoter. In these mice, expression of HPrP was detected only in brain tissue, with highest levels found in neurons of the cerebellum, hippocampus, thalamus, and cerebral cortex. These transgenic mice were susceptible to infection by the 263K strain of hamster scrapie with an average incubation period of 93 days, compared to 72 days in normal hamsters. In contrast, nontransgenic mice were not susceptible to this agent. These results indicate that neuron-specific expression of the 1 kb HPrP minigene including the HPrP open-reading frame is sufficient to mediate susceptibility to hamster scrapie, and that HPrP expression in nonneuronal brain cells is not necessary to overcome the TSE species barrier.

journal_name

Neuron

journal_title

Neuron

authors

Race RE,Priola SA,Bessen RA,Ernst D,Dockter J,Rall GF,Mucke L,Chesebro B,Oldstone MB

doi

10.1016/0896-6273(95)90105-1

subject

Has Abstract

pub_date

1995-11-01 00:00:00

pages

1183-91

issue

5

eissn

0896-6273

issn

1097-4199

pii

0896-6273(95)90105-1

journal_volume

15

pub_type

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