Prof Chris Shaw

Group Leader

Proteostatic mechanisms in FTD and ALS

Condition

Frontotemporal dementia, Motor neuron disease/ALS

Biography

A world-leader in the genetics of neurodegenerative diseases, Chris Shaw is Professor of Neurology & Neurogenetics at the IoPPN and Director of The Maurice Wohl Clinical Neuroscience Institute at Kings College London. After studying medicine in New Zealand, Chris did his doctoral training at the University of Cambridge as a Wellcome Fellow. A pioneer in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), since 2008 his group has discovered four new risk genes for ALS, and his work was recognised in 2019 with a Kea World Class New Zealand Award. Prof Shaw oversees an ambitious programme investigating proteostatic mechanisms and developing gene therapies for ALS and FTD. in 2021, he co-founded the UK DRI spin-out company Aviadobio to develop gene therapy treatments for FTD and ALS.

News

Key publications

Neurobiol Aging
Published

Striking phenotypic variation in a family with the P506S UBQLN2 mutation including amyotrophic lateral sclerosis, spastic paraplegia, and frontotemporal dementia.

Authors
Soragia Athina Gkazi, Claire Troakes, Simon Topp, Jack W Miller, Caroline A Vance, Jemeen Sreedharan, Ammar Al-Chalabi, Janine Kirby, Pamela J Shaw, Safa Al-Sarraj, Andrew King, Bradley N Smith, Christopher E Shaw
Striking phenotypic variation in a family with the P506S UBQLN2 mutation including amyotrophic lateral sclerosis, spastic paraplegia, and frontotemporal dementia.
Neurobiol Aging
Published

ALS-associated missense and nonsense TBK1 mutations can both cause loss of kinase function.

Authors
Martina de Majo, Simon D Topp, Bradley N Smith, Agnes L Nishimura, Han-Jou Chen, Athina Soragia Gkazi, Jack Miller, Chun Hao Wong, Caroline Vance, Frank Baas, Anneloor L M A Ten Asbroek, Kevin P Kenna, Nicola Ticozzi, Alberto Garcia Redondo, Jesús Esteban-Pérez, Cinzia Tiloca, Federico Verde, Stefano Duga, Karen E Morrison, Pamela J Shaw, Janine Kirby, Martin R Turner, Kevin Talbot, Orla Hardiman, Jonathan D Glass, Jacqueline de Belleroche, Cinzia Gellera, Antonia Ratti, Ammar Al-Chalabi, Robert H Brown, Vincenzo Silani, John E Landers, Christopher E Shaw
ALS-associated missense and nonsense TBK1 mutations can both cause loss of kinase function.
Nat Commun
Published

C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity.

Authors
Bhuvaneish T Selvaraj, Matthew R Livesey, Chen Zhao, Jenna M Gregory, Owain T James, Elaine M Cleary, Amit K Chouhan, Angus B Gane, Emma M Perkins, Owen Dando, Simon G Lillico, Youn-Bok Lee, Agnes L Nishimura, Urjana Poreci, Sai Thankamony, Meryll Pray, Navneet A Vasistha, Dario Magnani, Shyamanga Borooah, Karen Burr, David Story, Alexander McCampbell, Christopher E Shaw, Peter C Kind, Timothy J Aitman, C Bruce A Whitelaw, Ian Wilmut, Colin Smith, Gareth B Miles, Giles E Hardingham, David J A Wyllie, Siddharthan Chandran
C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity.
Hum Mol Genet
Published

C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.

Authors
Youn-Bok Lee, Pranetha Baskaran, Jorge Gomez-Deza, Han-Jou Chen, Agnes L Nishimura, Bradley N Smith, Claire Troakes, Yoshitsugu Adachi, Alan Stepto, Leonard Petrucelli, Jean-Marc Gallo, Frank Hirth, Boris Rogelj, Sarah Guthrie, Christopher E Shaw
C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.

Shaw Lab

Explore the work of the Shaw Lab, seeking to understand what causes amyotrophic lateral sclerosis and fronto-temporal dementia with the aim of finding more effective therapies

Shaw Lab