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 | 469 | 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 | 400 | 2014 |
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS M Prudencio, VV Belzil, R Batra, CA Ross, TF Gendron, LJ Pregent, ... Nature neuroscience 18 (8), 1175-1182, 2015 | 394 | 2015 |
Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood VV Belzil, PO Bauer, M Prudencio, TF Gendron, CT Stetler, IK Yan, ... Acta neuropathologica 126, 895-905, 2013 | 357 | 2013 |
Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease M Prudencio, PJ Hart, DR Borchelt, PM Andersen Human molecular genetics 18 (17), 3217-3226, 2009 | 299 | 2009 |
TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A XR Ma, M Prudencio, Y Koike, SC Vatsavayai, G Kim, F Harbinski, ... Nature 603 (7899), 124-130, 2022 | 294 | 2022 |
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 | 289 | 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 | 236 | 2017 |
Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS CM Karch, M Prudencio, DD Winkler, PJ Hart, DR Borchelt Proceedings of the National Academy of Sciences 106 (19), 7774-7779, 2009 | 236 | 2009 |
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 | 222 | 2019 |
A method to determine RNA and DNA oxidation simultaneously by HPLC-ECD: greater RNA than DNA oxidation in rat liver after doxorubicin administration T Hofer, AY Seo, M Prudencio, C Leeuwenburgh Walter de Gruyter 387 (1), 103-111, 2006 | 168 | 2006 |
Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia M Prudencio, J Humphrey, S Pickles, AL Brown, SE Hill, JM Kachergus, ... The Journal of clinical investigation 130 (11), 2020 | 153 | 2020 |
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 | 137 | 2016 |
Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis SV Seetharaman, M Prudencio, C Karch, SP Holloway, DR Borchelt, ... Experimental biology and medicine 234 (10), 1140-1154, 2009 | 122 | 2009 |
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 | 121 | 2014 |
Long-read sequencing across the C9orf72 ‘GGGGCC’ repeat expansion: implications for clinical use and genetic discovery efforts in human disease MTW Ebbert, SL Farrugia, JP Sens, K Jansen-West, TF Gendron, ... Molecular neurodegeneration 13, 1-17, 2018 | 118 | 2018 |
Repetitive element transcripts are elevated in the brain of C9orf72 ALS/FTLD patients M Prudencio, PK Gonzales, CN Cook, TF Gendron, LM Daughrity, Y Song, ... Human molecular genetics 26 (17), 3421-3431, 2017 | 113 | 2017 |
Phosphorylated neurofilament heavy chain: A biomarker of survival for C9ORF72‐associated amyotrophic lateral sclerosis TF Gendron, LM Daughrity, MG Heckman, NN Diehl, J Wuu, TM Miller, ... Annals of neurology 82 (1), 139-146, 2017 | 104 | 2017 |
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 | 101 | 2017 |
Misregulation of human sortilin splicing leads to the generation of a nonfunctional progranulin receptor M Prudencio, KR Jansen-West, WC Lee, TF Gendron, YJ Zhang, YF Xu, ... Proceedings of the National Academy of Sciences 109 (52), 21510-21515, 2012 | 99 | 2012 |