Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man LR Fischer, DG Culver, P Tennant, AA Davis, M Wang, ... Experimental neurology 185 (2), 232-240, 2004 | 1549 | 2004 |
Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis TF Gendron, J Chew, JN Stankowski, LR Hayes, YJ Zhang, M Prudencio, ... Science translational medicine 9 (383), eaai7866, 2017 | 249 | 2017 |
C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation W Cheng, S Wang, AA Mestre, C Fu, A Makarem, F Xian, LR Hayes, ... Nature communications 9 (1), 51, 2018 | 209 | 2018 |
Axonal degeneration in motor neuron disease LR Fischer, JD Glass Neurodegenerative Diseases 4 (6), 431-442, 2007 | 177 | 2007 |
SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse LR Fischer, A Igoudjil, J Magrané, Y Li, JM Hansen, G Manfredi, JD Glass Brain 134 (1), 196-209, 2011 | 149 | 2011 |
CRISPR-Cas9 screens identify the RNA helicase DDX3X as a repressor of C9ORF72 (GGGGCC) n repeat-associated non-AUG translation W Cheng, S Wang, Z Zhang, DW Morgens, LR Hayes, S Lee, B Portz, ... Neuron 104 (5), 885-898. e8, 2019 | 135 | 2019 |
G4C2 repeat RNA initiates a POM121-mediated reduction in specific nucleoporins in C9orf72 ALS/FTD AN Coyne, BL Zaepfel, L Hayes, B Fitchman, Y Salzberg, EC Luo, ... Neuron 107 (6), 1124-1140. e11, 2020 | 132 | 2020 |
The WldS gene modestly prolongs survival in the SOD1G93A fALS mouse LR Fischer, DG Culver, AA Davis, P Tennant, M Wang, M Coleman, ... Neurobiology of disease 19 (1-2), 293-300, 2005 | 130 | 2005 |
C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import LR Hayes, L Duan, K Bowen, P Kalab, JD Rothstein Elife 9, e51685, 2020 | 119 | 2020 |
Absence of SOD1 leads to oxidative stress in peripheral nerve and causes a progressive distal motor axonopathy LR Fischer, Y Li, SA Asress, DP Jones, JD Glass Experimental neurology 233 (1), 163-171, 2012 | 114 | 2012 |
Answer ALS, a large-scale resource for sporadic and familial ALS combining clinical and multi-omics data from induced pluripotent cell lines EG Baxi, T Thompson, J Li, JA Kaye, RG Lim, J Wu, D Ramamoorthy, ... Nature neuroscience 25 (2), 226-237, 2022 | 113 | 2022 |
Antibody therapy targeting RAN proteins rescues C9 ALS/FTD phenotypes in C9orf72 mouse model L Nguyen, F Montrasio, A Pattamatta, SK Tusi, O Bardhi, KD Meyer, ... Neuron 105 (4), 645-662. e11, 2020 | 97 | 2020 |
In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space A Igoudjil, J Magrané, LR Fischer, HJ Kim, I Hervias, M Dumont, C Cortez, ... Journal of Neuroscience 31 (44), 15826-15837, 2011 | 91 | 2011 |
Nuclear export and translation of circular repeat-containing intronic RNA in C9ORF72-ALS/FTD S Wang, MJ Latallo, Z Zhang, B Huang, DG Bobrovnikov, D Dong, ... Nature communications 12 (1), 4908, 2021 | 67 | 2021 |
Oxidative stress induced by loss of Cu, Zn-superoxide dismutase (SOD1) or superoxide-generating herbicides causes axonal degeneration in mouse DRG cultures LR Fischer, JD Glass Acta neuropathologica 119, 249-259, 2010 | 61 | 2010 |
Nuclear RNA binding regulates TDP-43 nuclear localization and passive nuclear export L Duan, BL Zaepfel, V Aksenova, M Dasso, JD Rothstein, P Kalab, ... Cell reports 40 (3), 2022 | 51 | 2022 |
A helicase unwinds hexanucleotide repeat RNA G-quadruplexes and facilitates repeat-associated non-AUG translation H Liu, YN Lu, T Paul, G Periz, MT Banco, AR Ferré-D’Amaré, JD Rothstein, ... Journal of the American Chemical Society 143 (19), 7368-7379, 2021 | 50 | 2021 |
Axonal degeneration in the peripheral nervous system: implications for the pathogenesis of amyotrophic lateral sclerosis LR Fischer-Hayes, T Brotherton, JD Glass Experimental neurology 246, 6-13, 2013 | 47 | 2013 |
Emerging therapies and novel targets for TDP-43 proteinopathy in ALS/FTD LR Hayes, P Kalab Neurotherapeutics 19 (4), 1061-1084, 2022 | 41 | 2022 |
An integrated multi-omic analysis of iPSC-derived motor neurons from C9ORF72 ALS patients H Phatnani, J Kwan, D Sareen, JR Broach, Z Simmons, X Arcila-Londono, ... Iscience 24 (11), 2021 | 35 | 2021 |