Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS TF Gendron, KF Bieniek, YJ Zhang, K Jansen-West, PEA Ash, T Caulfield, ... Acta neuropathologica 126, 829-844, 2013 | 643 | 2013 |
C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits J Chew, TF Gendron, M Prudencio, H Sasaguri, YJ Zhang, ... Science 348 (6239), 1151-1154, 2015 | 466 | 2015 |
Aggregation-prone c9FTD/ALS poly (GA) RAN-translated proteins cause neurotoxicity by inducing ER stress YJ Zhang, K Jansen-West, YF Xu, TF Gendron, KF Bieniek, WL Lin, ... Acta neuropathologica 128, 505-524, 2014 | 395 | 2014 |
Tau protein disrupts nucleocytoplasmic transport in Alzheimer’s disease B Eftekharzadeh, JG Daigle, LE Kapinos, A Coyne, J Schiantarelli, ... Neuron 99 (5), 925-940. e7, 2018 | 393 | 2018 |
Discovery of a biomarker and lead small molecules to target r (GGGGCC)-associated defects in c9FTD/ALS Z Su, Y Zhang, TF Gendron, PO Bauer, J Chew, WY Yang, E Fostvedt, ... Neuron 83 (5), 1043-1050, 2014 | 381 | 2014 |
C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins YJ Zhang, TF Gendron, JC Grima, H Sasaguri, K Jansen-West, YF Xu, ... Nature neuroscience 19 (5), 668-677, 2016 | 329 | 2016 |
Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis YJ Zhang, TF Gendron, MTW Ebbert, AD O’Raw, M Yue, K Jansen-West, ... Nature medicine 24 (8), 1136-1142, 2018 | 283 | 2018 |
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 | 234 | 2017 |
Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity YJ Zhang, L Guo, PK Gonzales, TF Gendron, Y Wu, K Jansen-West, ... Science 363 (6428), eaav2606, 2019 | 219 | 2019 |
Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy J Chew, C Cook, TF Gendron, K Jansen-West, G Del Rosso, ... Molecular neurodegeneration 14, 1-15, 2019 | 143 | 2019 |
Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts NJ Kramer, Y Carlomagno, YJ Zhang, S Almeida, CN Cook, TF Gendron, ... Science 353 (6300), 708-712, 2016 | 136 | 2016 |
Targeted manipulation of the sortilin–progranulin axis rescues progranulin haploinsufficiency WC Lee, S Almeida, M Prudencio, TR Caulfield, YJ Zhang, WM Tay, ... Human Molecular Genetics 23 (6), 1467-1478, 2014 | 116 | 2014 |
In-depth clinico-pathological examination of RNA foci in a large cohort of C9ORF72 expansion carriers M DeJesus-Hernandez, NCA Finch, X Wang, TF Gendron, KF Bieniek, ... Acta neuropathologica 134, 255-269, 2017 | 104 | 2017 |
Monitoring peripheral nerve degeneration in ALS by label-free stimulated Raman scattering imaging F Tian, W Yang, DA Mordes, JY Wang, JS Salameh, J Mok, J Chew, ... Nature communications 7 (1), 13283, 2016 | 103 | 2016 |
The lysosomal protein cathepsin L is a progranulin protease CW Lee, JN Stankowski, J Chew, CN Cook, YW Lam, S Almeida, ... Molecular neurodegeneration 12, 1-9, 2017 | 99 | 2017 |
MCT1 deletion in oligodendrocyte lineage cells causes late-onset hypomyelination and axonal degeneration T Philips, YA Mironova, Y Jouroukhin, J Chew, S Vidensky, MH Farah, ... Cell reports 34 (2), 2021 | 78 | 2021 |
Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD LD Goodman, M Prudencio, NJ Kramer, LF Martinez-Ramirez, ... Nature neuroscience 22 (6), 863-874, 2019 | 69 | 2019 |
Molecularly defined cortical astroglia subpopulation modulates neurons via secretion of Norrin SJ Miller, T Philips, N Kim, R Dastgheyb, Z Chen, YC Hsieh, JG Daigle, ... Nature neuroscience 22 (5), 741-752, 2019 | 69 | 2019 |
ADAR2 mislocalization and widespread RNA editing aberrations in C9orf72-mediated ALS/FTD S Moore, E Alsop, I Lorenzini, A Starr, BE Rabichow, E Mendez, JL Levy, ... Acta neuropathologica 138, 49-65, 2019 | 66 | 2019 |
The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo H Sasaguri, J Chew, YF Xu, TF Gendron, A Garrett, CW Lee, ... Brain research 1647, 57-64, 2016 | 58 | 2016 |