CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia KL Williams, S Topp, S Yang, B Smith, JA Fifita, ST Warraich, KY Zhang, ... Nature communications 7 (1), 11253, 2016 | 229 | 2016 |
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions NE Farrawell, IA Lambert-Smith, ST Warraich, IP Blair, DN Saunders, ... Scientific reports 5 (1), 13416, 2015 | 147 | 2015 |
Proteome homeostasis dysfunction: a unifying principle in ALS pathogenesis JJ Yerbury, NE Farrawell, L McAlary Trends in Neurosciences 43 (5), 274-284, 2020 | 58 | 2020 |
Tryptophan 32-mediated SOD1 aggregation is attenuated by pyrimidine-like compounds in living cells E Pokrishevsky, L McAlary, NE Farrawell, B Zhao, M Sher, JJ Yerbury, ... Scientific Reports 8 (1), 15590, 2018 | 46 | 2018 |
SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS NE Farrawell, I Lambert-Smith, K Mitchell, J McKenna, L McAlary, ... Journal of Cell Science 131 (11), jcs209122, 2018 | 46 | 2018 |
SerpinB2 (PAI-2) modulates proteostasis via binding misfolded proteins and promotion of cytoprotective inclusion formation JA Lee, JJ Yerbury, N Farrawell, RF Shearer, P Constantinescu, ... PLoS One 10 (6), e0130136, 2015 | 45 | 2015 |
The ubiquitin proteasome system is a key regulator of pluripotent stem cell survival and motor neuron differentiation M Bax, J McKenna, D Do-Ha, CH Stevens, S Higginbottom, R Balez, ... Cells 8 (6), 581, 2019 | 42 | 2019 |
Ubiquitin homeostasis is disrupted in TDP-43 and FUS cell models of ALS NE Farrawell, L McAlary, JS Lum, CG Chisholm, ST Warraich, IP Blair, ... Iscience 23 (11), 2020 | 33 | 2020 |
Alpha-2-macroglobulin is acutely sensitive to freezing and lyophilization: Implications for structural and functional studies AR Wyatt, JR Kumita, NE Farrawell, CM Dobson, MR Wilson PLoS One 10 (6), e0130036, 2015 | 31 | 2015 |
Flow cytometric measurement of the cellular propagation of TDP-43 aggregation R Zeineddine, DR Whiten, NE Farrawell, L McAlary, MA Hanspal, ... Prion 11 (3), 195-204, 2017 | 30 | 2017 |
p62 overexpression induces TDP-43 cytoplasmic mislocalisation, aggregation and cleavage and neuronal death AD Foster, LL Flynn, C Cluning, F Cheng, JM Davidson, A Lee, N Polain, ... Scientific reports 11 (1), 11474, 2021 | 26 | 2021 |
Addition of exogenous SOD1 aggregates causes TDP-43 mislocalisation and aggregation R Zeineddine, NE Farrawell, IA Lambert-Smith, JJ Yerbury Cell Stress and Chaperones 22 (6), 893-902, 2017 | 26 | 2017 |
CuATSM Protects Against the In Vitro Cytotoxicity of Wild-Type-Like Copper–Zinc Superoxide Dismutase Mutants but not Mutants That Disrupt Metal Binding NE Farrawell, MR Yerbury, SS Plotkin, L McAlary, JJ Yerbury ACS chemical neuroscience 10 (3), 1555-1564, 2018 | 21 | 2018 |
TDP-43 is a ubiquitylation substrate of the SCFcyclin F complex SL Rayner, S Yang, NE Farrawell, CJ Jagaraj, F Cheng, JM Davidson, ... Neurobiology of disease 167, 105673, 2022 | 13 | 2022 |
Ubiquitin homeostasis disruption, a common cause of proteostasis collapse in amyotrophic lateral sclerosis? CG Chisholm, JS Lum, NE Farrawell, JJ Yerbury Neural Regeneration Research 17 (10), 2218-2220, 2022 | 10 | 2022 |
Mutant Cu/Zn superoxide dismutase (A4V) turnover is altered in cells containing inclusions NE Farrawell, JJ Yerbury Frontiers in molecular neuroscience 14, 771911, 2021 | 9 | 2021 |
CuATSM improves motor function and extends survival but is not tolerated at a high dose in SOD1G93A mice with a C57BL/6 background JS Lum, ML Brown, NE Farrawell, L McAlary, D Ly, CG Chisholm, J Snow, ... Scientific Reports 11 (1), 19392, 2021 | 9 | 2021 |
CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia. Nat Commun 7: 11253 KL Williams, S Topp, S Yang, B Smith, JA Fifita, ST Warraich, KY Zhang, ... | 7 | 2016 |
ALS-linked CCNF variant disrupts motor neuron ubiquitin homeostasis NE Farrawell, M Bax, L McAlary, J McKenna, S Maksour, D Do-Ha, ... Human Molecular Genetics 32 (14), 2386-2398, 2023 | 3 | 2023 |
Cells Overexpressing ALS-Associated SOD1 Variants Are Differentially Susceptible to CuATSM-Associated Toxicity ML Brown, L McAlary, JS Lum, NE Farrawell, JJ Yerbury ACS Chemical Neuroscience 13 (16), 2371-2379, 2022 | 1 | 2022 |