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TDP-43 triggers mitochondrial DNA release via mPTP to activate cGAS/STING in ALS CH Yu, S Davidson, CR Harapas, JB Hilton, MJ Mlodzianoski, ... Cell 183 (3), 636-649. e18, 2020 | 662 | 2020 |
Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS BJ Turner, K Talbot Progress in neurobiology 85 (1), 94-134, 2008 | 621 | 2008 |
Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and-independent mechanisms LI Grad, JJ Yerbury, BJ Turner, WC Guest, E Pokrishevsky, MA O’Neill, ... Proceedings of the National Academy of Sciences 111 (9), 3620-3625, 2014 | 473 | 2014 |
Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1 JD Atkin, MA Farg, BJ Turner, D Tomas, JA Lysaght, J Nunan, A Rembach, ... Journal of Biological Chemistry 281 (40), 30152-30165, 2006 | 360 | 2006 |
Emerging therapies and challenges in spinal muscular atrophy MA Farrar, SB Park, S Vucic, KA Carey, BJ Turner, TH Gillingwater, ... Annals of neurology 81 (3), 355-368, 2017 | 279 | 2017 |
Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis BJ Turner, JD Atkin, MA Farg, A Rembach, EC Lopes, JD Patch, AF Hill, ... Journal of Neuroscience 25 (1), 108-117, 2005 | 233 | 2005 |
The bone morphogenetic protein axis is a positive regulator of skeletal muscle mass CE Winbanks, JL Chen, H Qian, Y Liu, BC Bernardo, C Beyer, KI Watt, ... Journal of Cell Biology 203 (2), 345-357, 2013 | 230 | 2013 |
ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation AK Walker, KY Soo, V Sundaramoorthy, S Parakh, Y Ma, MA Farg, ... PloS one 8 (11), e81170, 2013 | 204 | 2013 |
Oral treatment with CuII (atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis BR Roberts, NKH Lim, EJ McAllum, PS Donnelly, DJ Hare, PA Doble, ... Journal of Neuroscience 34 (23), 8021-8031, 2014 | 184 | 2014 |
The Hippo pathway effector YAP is a critical regulator of skeletal muscle fibre size KI Watt, BJ Turner, A Hagg, X Zhang, JR Davey, H Qian, C Beyer, ... Nature communications 6 (1), 6048, 2015 | 174 | 2015 |
Association of regulatory T-cell expansion with progression of amyotrophic lateral sclerosis: a study of humans and a transgenic mouse model RK Sheean, FC McKay, E Cretney, CR Bye, ND Perera, D Tomas, ... JAMA neurology 75 (6), 681-689, 2018 | 154 | 2018 |
Diacetylbis (N (4)-methylthiosemicarbazonato) copper (II)(CuII (atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral … CPW Soon, PS Donnelly, BJ Turner, LW Hung, PJ Crouch, NA Sherratt, ... Journal of Biological Chemistry 286 (51), 44035-44044, 2011 | 153 | 2011 |
Antisense peptide nucleic acid‐mediated knockdown of the p75 neurotrophin receptor delays motor neuron disease in mutant SOD1 transgenic mice BJ Turner, IK Cheah, KJ Macfarlane, EC Lopes, S Petratos, SJ Langford, ... Journal of neurochemistry 87 (3), 752-763, 2003 | 132 | 2003 |
Disease mechanisms in ALS: misfolded SOD1 transferred through exosome-dependent and exosome-independent pathways JM Silverman, SM Fernando, LI Grad, AF Hill, BJ Turner, JJ Yerbury, ... Cellular and molecular neurobiology 36, 377-381, 2016 | 122 | 2016 |
SYT1-associated neurodevelopmental disorder: a case series K Baker, SL Gordon, H Melland, F Bumbak, DJ Scott, TJ Jiang, D Owen, ... Brain 141 (9), 2576-2591, 2018 | 121 | 2018 |
Ferroptosis mediates selective motor neuron death in amyotrophic lateral sclerosis T Wang, D Tomas, ND Perera, B Cuic, S Luikinga, A Viden, SK Barton, ... Cell Death & Differentiation 29 (6), 1187-1198, 2022 | 100 | 2022 |
Perturbed BMP signaling and denervation promote muscle wasting in cancer cachexia R Sartori, A Hagg, S Zampieri, A Armani, CE Winbanks, LR Viana, ... Science translational medicine 13 (605), eaay9592, 2021 | 95 | 2021 |
Mutant SOD 1 inhibits ER‐Golgi transport in amyotrophic lateral sclerosis JD Atkin, MA Farg, KY Soo, AK Walker, M Halloran, BJ Turner, P Nagley, ... Journal of neurochemistry 129 (1), 190-204, 2014 | 94 | 2014 |
SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation R Zeineddine, JF Pundavela, L Corcoran, EM Stewart, D Do-Ha, M Bax, ... Molecular neurodegeneration 10, 1-18, 2015 | 93 | 2015 |