The University of Southampton
University of Southampton Institutional Repository

Neuropathologically distinct prion strains give rise to similar temporal profiles of behavioral deficits

Neuropathologically distinct prion strains give rise to similar temporal profiles of behavioral deficits
Neuropathologically distinct prion strains give rise to similar temporal profiles of behavioral deficits
Mouse-adapted scrapie strains have been characterized by vacuolation profiles and incubation times, but the behavioral consequences have not been well studied. Here, we compared behavioral impairments produced by ME7, 79A, 22L, and 22A strains in C57BL/6J mice. We show that early impairments on burrowing, glucose consumption, nesting and open field activity, and late stage motor impairments show a very similar temporal sequence in ME7, 79A, and 22L. The long incubation time of the 22A strain produces much later impairments. However, the strains show clear late stage neuropathological differences. All strains showed clear microglial activation and synaptic loss in the hippocampus, but only ME7 and 79A showed significant CA1 neuronal death. Conversely, 22L and 22A showed significant cerebellar Purkinje neuron loss. All strains showed marked thalamic neuronal loss. These behavioral similarities coupled with clear pathological differences could serve to identify key circuits whose early dysfunction underlies the neurological effects of different prion strains
behavior, neurodegeneration, scrapie, dysfunction, mouse, strains, prion, thalamus, hippocampus, cerebellum
0969-9961
258-269
Cunningham, C.
6d675038-a4b1-46e2-9e4b-0a5ac27ea2b2
Deacon, RMJ.
7898c2bc-8a93-4da4-afbc-ab19c01de578
Chan, K.
2fad0805-393b-4c6c-90a2-fd6fd9dfa29c
Boche, D.
bdcca10e-6302-4dd0-919f-67218f7e0d61
Rawlins, JNP.
383e8cd4-7925-4cab-aa3b-658ecbab0bf0
Perry, VH.
8f29d36a-8e1f-4082-8700-09483bbaeae4
Cunningham, C.
6d675038-a4b1-46e2-9e4b-0a5ac27ea2b2
Deacon, RMJ.
7898c2bc-8a93-4da4-afbc-ab19c01de578
Chan, K.
2fad0805-393b-4c6c-90a2-fd6fd9dfa29c
Boche, D.
bdcca10e-6302-4dd0-919f-67218f7e0d61
Rawlins, JNP.
383e8cd4-7925-4cab-aa3b-658ecbab0bf0
Perry, VH.
8f29d36a-8e1f-4082-8700-09483bbaeae4

Cunningham, C., Deacon, RMJ., Chan, K., Boche, D., Rawlins, JNP. and Perry, VH. (2005) Neuropathologically distinct prion strains give rise to similar temporal profiles of behavioral deficits. Neurobiology of Disease, 18 (2), 258-269. (doi:10.1016/j.nbd.2004.08.015).

Record type: Article

Abstract

Mouse-adapted scrapie strains have been characterized by vacuolation profiles and incubation times, but the behavioral consequences have not been well studied. Here, we compared behavioral impairments produced by ME7, 79A, 22L, and 22A strains in C57BL/6J mice. We show that early impairments on burrowing, glucose consumption, nesting and open field activity, and late stage motor impairments show a very similar temporal sequence in ME7, 79A, and 22L. The long incubation time of the 22A strain produces much later impairments. However, the strains show clear late stage neuropathological differences. All strains showed clear microglial activation and synaptic loss in the hippocampus, but only ME7 and 79A showed significant CA1 neuronal death. Conversely, 22L and 22A showed significant cerebellar Purkinje neuron loss. All strains showed marked thalamic neuronal loss. These behavioral similarities coupled with clear pathological differences could serve to identify key circuits whose early dysfunction underlies the neurological effects of different prion strains

Full text not available from this repository.

More information

Published date: March 2005
Keywords: behavior, neurodegeneration, scrapie, dysfunction, mouse, strains, prion, thalamus, hippocampus, cerebellum

Identifiers

Local EPrints ID: 27558
URI: https://eprints.soton.ac.uk/id/eprint/27558
ISSN: 0969-9961
PURE UUID: bff95854-a80e-4fcd-99ce-56abec90ca42
ORCID for D. Boche: ORCID iD orcid.org/0000-0002-5884-130X

Catalogue record

Date deposited: 25 Apr 2006
Last modified: 06 Jun 2018 12:52

Export record

Altmetrics

Download statistics

Downloads from ePrints over the past year. Other digital versions may also be available to download e.g. from the publisher's website.

View more statistics

Atom RSS 1.0 RSS 2.0

Contact ePrints Soton: eprints@soton.ac.uk

ePrints Soton supports OAI 2.0 with a base URL of https://eprints.soton.ac.uk/cgi/oai2

This repository has been built using EPrints software, developed at the University of Southampton, but available to everyone to use.

We use cookies to ensure that we give you the best experience on our website. If you continue without changing your settings, we will assume that you are happy to receive cookies on the University of Southampton website.

×