NAT10 inhibition corrects nuclear defects in tau mutant human neurons and extends lifespan in a Drosophila tauopathy model
iScience 2026-08
DOI: 10.1016/j.isci.2026.116861
Francesco Paonessa; Bernardo Delarue Bizzini; Tom Campbell; Emily Coode; Jonathan Lam; Ravi Solanki; Richard Butler; James Smith; Catherine M. Davidson; Delphine Larrieu et al.
A phenotypic screen for novel small molecules that correct tau-mediated pathologies in human frontotemporal dementia neurons
Alzheimer's & Dementia 2025-09
DOI: 10.1002/alz.70620
Hannah Maxwell; James Smith; Tom Campbell; Jason Clark; Rosanna La Rocca; Francesco Paonessa; Sergey Sitnikov; Mark Evans; Peter Maycox; Frederick J. Livesey
Tau uptake by human neurons depends on receptor LRP1 and kinase LRRK2
The EMBO Journal 2025-09-15
DOI: 10.1038/s44318-025-00514-0
Lewis D Evans; Alessio Strano; Eleanor Tuck; Ashley Campbell; James Smith; Christy Hung; Tiana S Behr; Bernardino Ghetti; Benjamin Ryskeldi-Falcon; Emre Karakoc et al.
SORL1 deficiency in human excitatory neurons causes APP-dependent defects in the endolysosome-autophagy network
Cell Reports 2021-06
DOI: 10.1016/j.celrep.2021.109259 PMID: 34133918 PMC: PMC8220253
Christy Hung; Eleanor Tuck; Victoria Stubbs; Sven J van der Lee; Cora Aalfs; Resie van Spaendonk; Philip Scheltens; John Hardy; Henne Holstege; Frederick J Livesey
Microtubules Deform the Nuclear Membrane and Disrupt Nucleocytoplasmic Transport in Tau-Mediated Frontotemporal Dementia
Cell Reports 2019
DOI: 10.1016/j.celrep.2018.12.085
Francesco Paonessa; Lewis D. Evans; Ravi Solanki; Delphine Larrieu; Selina Wray; John Hardy; Stephen P. Jackson; Frederick J. Livesey
In vivo modeling of human neuron dynamics and down syndrome
Science 2018
DOI: 10.1126/science.aau1810
Raquel Real; Manuel Peter; Antonio Trabalza; Shabana Khan; Mark A. Smith; Joana Dopp; Samuel J. Barnes; Ayiba Momoh; Alessio Strano; Emanuela Volpi; Graham Knott; Frederick J. Livesey; Vincenzo De Paola
Altered γ-Secretase Processing of APP Disrupts Lysosome and Autophagosome Function in Monogenic Alzheimer's Disease
Cell Reports 2018-12
DOI: 10.1016/j.celrep.2018.11.095 PMID: 30590039 PMC: PMC6315085
Christy O Y Hung; Frederick J Livesey
Functional Studies of Missense TREM2 Mutations in Human Stem Cell-Derived Microglia
Stem Cell Reports 2018-03
DOI: 10.1016/j.stemcr.2018.03.003 PMID: 29606617
Philip W Brownjohn; James Smith; Ravi Solanki; Ebba Lohmann; Henry Houlden; John Hardy; Sabine Dietmann; Frederick J Livesey
Phenotypic Screening Identifies Modulators of Amyloid Precursor Protein Processing in Human Stem Cell Models of Alzheimer's Disease
Stem Cell Reports 2017-04
DOI: 10.1016/j.stemcr.2017.02.006 PMID: 28285880 PMC: PMC5390098
Philip W Brownjohn; James Smith; Erik Portelius; Lutgarde Serneels; Hlin Kvartsberg; Bart De Strooper; Kaj Blennow; Henrik Zetterberg; Frederick J Livesey
APP metabolism regulates tau proteostasis in human cerebral cortex neurons
Cell Reports 2015-05
DOI: 10.1016/j.celrep.2015.03.068 PMID: 25921538 PMC: PMC4431668
Steven Moore; Lewis D.B. Evans; Therese Andersson; Erik Portelius; James Smith; Tatyana B. Dias; Nathalie Saurat; Amelia McGlade; Peter Kirwan; Kaj Blennow; John Hardy; Henrik Zetterberg; Frederick J. Livesey
Human cerebral cortex development from pluripotent stem cells to functional excitatory synapses
Nature Neuroscience 2012-03
DOI: 10.1038/nn.3041 PMID: 22306606 PMC: PMC3882590
Yichen Shi; Peter Kirwan; James Smith; Hugh P C Robinson; Frederick J Livesey
A human stem cell model of early Alzheimer's disease pathology in Down syndrome
Science Translational Medicine 2012-03
DOI: 10.1126/scitranslmed.3003771 PMID: 22344463 PMC: PMC4129935
Yichen Shi; Peter Kirwan; James Smith; Glenn MacLean; Stuart H. Orkin; Frederick J. Livesey