Journal article

Tailored behavioural tests reveal early and progressive cognitive deficits in M1000 prion disease

M Senesi, V Lewis, PA Adlard, DI Finkelstein, JH Kim, SJ Collins

Neurobiology of Disease | Published : 2023

Abstract

Prion diseases are pathogenically linked to the normal cellular prion protein (PrPC) misfolding into abnormal conformers (PrPSc), with PrPSc accumulation underpinning both transmission and neurotoxicity. Despite achieving this canonical understanding, however fundamental questions remain incompletely resolved, including the level of pathophysiological overlap between neurotoxic and transmitting species of PrPSc and the temporal profiles of their propagation. To further investigate the likely time of occurrence of significant levels of neurotoxic species during prion disease development, the well characterised in vivo M1000 murine model was employed. Following intracerebral inoculation, detai..

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Grants

Awarded by Australian Research Council


Funding Acknowledgements

VL has been supported by the CJD Support Group Network; MS has been supported by The University of Melbourne ?Melbourne Interna- tional Research Scholarship? and by a CJD Support Group Network ?In memory of Silva Coelho? travel grant; PAA is supported by an ARC Future Fellowship; SJC is supported in part by a NHMRC Practitioner Fellowship #APP1105784, JHK is supported by the NHMRC Career Development Fellowship #APP1083309. The Florey Department of Neuroscience and Mental Health acknowledges the strong support from the Victorian Government and the funding from the Operational Infra- structure Support Grant.