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MRC Prion Unit and Institute of Prion diseases

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Tom Cunningham

Mouse Models of Prion Disease
Tom

t.cunningham@prion.ucl.ac.uk
tom.cunningham@ucl.ac.uk
Tel: 020 7679 5024
Courtauld Building, Room 101A

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Research Synopsis听

Using genome engineering technologies in mice, we aim to understand the molecular and phenotypic nature of prion strains as well as provide models for evaluating candidate small molecule and antibody therapeutics, which we anticipate will have relevance for treating other neurodegenerative diseases. Modelling inherited prion disease, caused by genetic mutations in the gene encoding the prion protein, constitutes 10-15% human prion disease cases, and provides an opportunity to model and study diverse strain types and characteristics that may illuminate wider mechanisms of prion biology, and wider mechanisms of neurodegeneration. We generate and study transgenic and knock-in humanised models in order to maintain authentic human strain characteristics that have been proven to be distinct from prions derived from other mammalian species.

EA Fig 1

Selected Publications


Devoy A, Price G, De Giorgio F, Bunton-Stasyshyn R, Thompson D, Gasco S, Allan A, Codner GF, Nair RR, Tibbit C, McLeod R, Ali Z, Noda J, Marrero-Gagliardi A, Brito-Armas JM, Williams C, Ozturk M, Simon M, O鈥橬eill E, Harrison J, Atkins G, Corrochano S, Stewart M, Gilthorpe JD, Teboul L, Acevedo-Arozena A 搂 , Fisher EMC and Cunningham TJ. iScience. 2021 Nov 15;24(12):103463.


Garone MG, Birsa N, Rosito M, Salaris F, Mochi M, de Turris V, Nair RR, Cunningham TJ, Fisher EMC, Morlando M, Fratta P, Rosa A. Commun Biol. 2021 Sep 1;4(1):1025.


Nair RR, Tibbit C, Thompson D, McLeod R, Nakhuda A, Simon MM, Baloh RH, Fisher EMC, Isaacs AM, Cunningham TJ.Methods. 2020; Jul 25;S1046-2023(20)30028-1.


Maza AM, Jarvis S, Lee WC, Cunningham TJ, Schiavo G, Secrier M, Fratta P, Sleigh JN, Sudre CH, Fisher EMC, Sudre CH.听 Scientific Reports. 2021;11: 12251. Cunningham TJ, Fisher EMC, Fratta P, Gilthorpe JD. DNA Editing for Amyotrophic Lateral Sclerosis: Leading Off First Base. CRISPR J. 2020; Apr;3(2):75-77.

Zhu F, Nair RR, Fisher EMC搂 , Cunningham TJ搂 .听 Nat Commun. 2019;10(1):1845.


Nair RR, Corrochano S, Gasco S, Tibbit C, Thompson D, Maduro C, Ali Z, Acevedo-Arozena A, Cunningham TJ搂 , Fisher EMC搂 .听 Mamm Genome. 2019;30(7-8):173-191.


Sivakumar P, De Giorgio F, Ule AM, Neeves J, Nair RR, Bentham M, Birsa N, Humphrey J, Plagnol V, Acevedo-Arozena A, Cunningham TJ, Fisher EMC, Fratta P. . Brain. 2018;141(12):e83