Professor Peter St George-Hyslop

University Position

Professor

Interests

My laboratory focuses upon understanding the causes and molecular mechanisms of neurodegenerative diseases such as Alzheimer's Disease, Parkinson's Disease and Fronto-Temporal Dementia. We and others have shown that these diseases are frequently caused by the accumulation neurotoxic proteins or protein fragments. We employ genetic, molecular biological, cell biological, and animal modeling strategies to: 1) identify disease-causing genes; and 2) to identify the molecular pathways by which these mutation or polymorphisms lead to neuronal death.

Key Publications

Mutation of the ALS/FTD-associated RNA-binding protein FUS affects axonal development.

DOI: http://doi.org/10.1523/JNEUROSCI.2148-23.2024
Journal: J Neurosci
E-pub date: 3 Jul 2024
Authors: FWV Tartwijk, LCS Wunderlich, I Mela, S Makarchuk, MAH Jakobs, S Qamar, K Franze, GSK Schierle, PHS George-Hyslop, JQ Lin, CE Holt, CF Kaminski

Phase Separation of FUS is Modulated by Methylation State of Cation-π Interactions and Interaction with TNPO1

DOI: http://doi.org/10.1016/j.cell.2018.03.056
Journal: Cell
E-pub date: 19 Apr 2018
Authors: PH St George-Hyslop, S Qamar, G Wang, SJ Randle, FS Ruggeri, J Varela, CF Kaminski, GS Kaminski, M Vendruscolo, TPJ Knowles, D Klenerman, CE Holt, Q Lin, W meadows

ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function.

DOI: http://doi.org/10.1016/j.neuron.2015.10.030
Journal: Neuron
E-pub date: 18 Nov 2015
Authors: T Murakami, S Qamar, JQ Lin, GSK Schierle, E Rees, A Miyashita, AR Costa, RB Dodd, FTS Chan, CH Michel, D Kronenberg-Versteeg, Y Li, S-P Yang, Y Wakutani, W Meadows, RR Ferry, L Dong, GG Tartaglia, G Favrin, W-L Lin, DW Dickson, M Zhen, D Ron, G Schmitt-Ulms, PE Fraser, NA Shneider, C Holt, M Vendruscolo, CF Kaminski, P St George-Hyslop

Publications

Z-α1-antitrypsin polymers impose molecular filtration in the endoplasmic reticulum after undergoing phase transition to a solid state.

DOI: http://doi.org/10.1126/sciadv.abm2094
Journal: Sci Adv
E-pub date: 8 Apr 2022
Authors: JE Chambers, N Zubkov, M Kubánková, J Nixon-Abell, I Mela, S Abreu, M Schwiening, G Lavarda, I López-Duarte, JA Dickens, T Torres, CF Kaminski, LJ Holt, E Avezov, JA Huntington, PS George-Hyslop, MK Kuimova, SJ Marciniak

Does Soluble TREM2 Protect Against Alzheimer’s Disease?

DOI: http://doi.org/10.3389/fnagi.2021.834697
Journal: Front Aging Neurosci
E-pub date: 1 Aug 2021
Authors: GC Brown, P St George-Hyslop

TET2 Regulates the Neuroinflammatory Response in Microglia.

DOI: http://doi.org/10.1016/j.celrep.2019.09.013
Journal: Cell Rep
E-pub date: 15 Oct 2019
Authors: A Carrillo-Jimenez, Ö Deniz, MV Niklison-Chirou, R Ruiz, K Bezerra-Salomão, V Stratoulias, R Amouroux, PK Yip, A Vilalta, M Cheray, AM Scott-Egerton, E Rivas, K Tayara, I García-Domínguez, J Garcia-Revilla, JC Fernandez-Martin, AM Espinosa-Oliva, X Shen, P St George-Hyslop, GC Brown, P Hajkova, B Joseph, JL Venero, MR Branco, MA Burguillos

The physiological and pathological biophysics of phase separation and gelation of RNA binding proteins in amyotrophic lateral sclerosis and fronto-temporal lobar degeneration.

DOI: http://doi.org/10.1016/j.brainres.2018.04.036
Journal: Brain Res
E-pub date: 15 Aug 2018
Authors: P St George-Hyslop, JQ Lin, A Miyashita, EC Phillips, S Qamar, SJ Randle, G Wang