Zobrazeno 1 - 10
of 52
pro vyhledávání: '"Richard J Mead"'
Publikováno v:
Nature Communications, Vol 15, Iss 1, Pp 1-11 (2024)
Abstract Amyotrophic lateral sclerosis (ALS) is characterized by degeneration of motor neurons in the central nervous system (CNS). Mutations in the metalloenzyme SOD1 are associated with inherited forms of ALS and cause a toxic gain of function thou
Externí odkaz:
https://doaj.org/article/43dd0b8021d242968a9526c2e1593cad
Publikováno v:
PLoS ONE, Vol 9, Iss 9, p e107918 (2014)
The SOD1G93A mouse has been used since 1994 for preclinical testing in amyotrophic lateral sclerosis (ALS). Despite recent genetic advances in our understanding of ALS, transgenic mice expressing mutant SOD1 remain the best available, and most widely
Externí odkaz:
https://doaj.org/article/b709dfb8daa244d18969cc921db5730c
Autor:
Richard J Mead, Ellen J Bennett, Aneurin J Kennerley, Paul Sharp, Claire Sunyach, Paul Kasher, Jason Berwick, Brigitte Pettmann, Guiseppe Battaglia, Mimoun Azzouz, Andrew Grierson, Pamela J Shaw
Publikováno v:
PLoS ONE, Vol 6, Iss 8, p e23244 (2011)
The human SOD1(G93A) transgenic mouse has been used extensively since its development in 1994 as a model for amyotrophic lateral sclerosis (ALS). In that time, a great many insights into the toxicity of mutant SOD1 have been gained using this and oth
Externí odkaz:
https://doaj.org/article/0a8e58065d064ae8ae3e91567ca6ba0c
Autor:
Peter J. Richardson, Daniel P. Smith, Alex de Giorgio, Xenia Snetkov, Joshua Almond-Thynne, Sara Cronin, Richard J. Mead, Christopher J. McDermott, Pamela J. Shaw
Publikováno v:
Translational Neurodegeneration, Vol 12, Iss 1, Pp 1-17 (2023)
Abstract Amyotrophic lateral sclerosis (ALS) is a poorly treated multifactorial neurodegenerative disease associated with multiple cell types and subcellular organelles. As with other multifactorial diseases, it is likely that drugs will need to targ
Externí odkaz:
https://doaj.org/article/f1de506708b2457081b6e57e8800b184
Autor:
Scott P. Allen, Afnan Al Sultan, Elaine Kabucho Kibirige, Erin Tonkiss, Keaton J. Hamer, Lydia M. Castelli, Ya-Hui Lin, Sarah Roscoe, Nikolaos Stefanidis, Richard J. Mead, J. Robin Highley, Johnathan Cooper-Knock, Guillaume M. Hautbergue, Paul R. Heath, Janine Kirby, Pamela J. Shaw
Publikováno v:
Frontiers in Aging Neuroscience, Vol 15 (2023)
A p.Y374X truncation in TARDBP was recently shown to reduce expression of TDP43 in fibroblasts isolated from ALS cases. In this follow up study focused on assessing the downstream phenotypic consequences of loss of TDP43 in the context of the truncat
Externí odkaz:
https://doaj.org/article/ca2ad4e34a5b4061a9d5da2449950735
Autor:
James J. P. Alix, Maria Plesia, Chlöe N. Schooling, Alexander P. Dudgeon, Catherine A. Kendall, Visakan Kadirkamanathan, Christopher J. McDermott, Gráinne S. Gorman, Robert W. Taylor, Richard J. Mead, Pamela J. Shaw, John C. Day
Publikováno v:
Journal of Raman Spectroscopy. 54:258-268
Publikováno v:
Nature Reviews Drug Discovery. 22:185-212
Autor:
Lydia M. Castelli, Ya-Hui Lin, Alvaro Sanchez-Martinez, Aytaç Gül, Kamallia Mohd Imran, Adrian Higginbottom, Santosh Kumar Upadhyay, Nóra M. Márkus, Raquel Rua Martins, Johnathan Cooper-Knock, Claire Montmasson, Rebecca Cohen, Amy Walton, Claudia S. Bauer, Kurt J. De Vos, Richard J. Mead, Mimoun Azzouz, Cyril Dominguez, Laura Ferraiuolo, Pamela J. Shaw, Alexander J. Whitworth, Guillaume M. Hautbergue
Publikováno v:
Science Translational Medicine. 15
Hexanucleotide repeat expansions in C9ORF72 are the most common genetic cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Studies have shown that the hexanucleotide expansions cause the noncanonical translation
Autor:
Laura Ferraiuolo, Noemi Gatto, Andrew J. Grierson, Chloe F. Allen, Richard J. Mead, Yuri Ciervo, Pamela J. Shaw
Publikováno v:
Molecular Therapy: Methods & Clinical Development, Vol 21, Iss, Pp 413-433 (2021)
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative condition for which new therapeutic options are urgently needed. We injected GFP+ adipose-derived stem cells (EGFP-ADSCs) directly into the cerebrospinal fluid (CSF) of transgenic
Autor:
Oliver A C Stevens, Gavin R. Lloyd, Maria Plesia, Richard J. Mead, John C. Day, Pamela J. Shaw, Gaynor Miller, James J.P. Alix, Ian Coldicott, Aneurin J. Kennerley, Catherine Kendall
Publikováno v:
ACS Chemical Neuroscience
Plesia, M, Stevens, O A, Lloyd, G R, Kendall, C A, Coldicott, I, Kennerley, A J, Miller, G, Shaw, P J, Mead, R J, Day, J C C & Alix, J J P 2021, ' In Vivo Fiber Optic Raman Spectroscopy of Muscle in Preclinical Models of Amyotrophic Lateral Sclerosis and Duchenne Muscular Dystrophy ', ACS Chemical Neuroscience, vol. 12, no. 10, pp. 1768-1776 . https://doi.org/10.1021/acschemneuro.0c00794
Plesia, M, Stevens, O A, Lloyd, G R, Kendall, C A, Coldicott, I, Kennerley, A J, Miller, G, Shaw, P J, Mead, R J, Day, J C C & Alix, J J P 2021, ' In Vivo Fiber Optic Raman Spectroscopy of Muscle in Preclinical Models of Amyotrophic Lateral Sclerosis and Duchenne Muscular Dystrophy ', ACS Chemical Neuroscience, vol. 12, no. 10, pp. 1768-1776 . https://doi.org/10.1021/acschemneuro.0c00794
Neuromuscular diseases result in muscle weakness, disability, and, in many instances, death. Preclinical models form the bedrock of research into these disorders, and the development of in vivo and potentially translational biomarkers for the accurat