| 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 | 482 | 2015 |
| 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 | 395 | 2014 |
| 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 | 369 | 2013 |
| Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study M van Blitterswijk, M DeJesus-Hernandez, E Niemantsverdriet, ... The Lancet Neurology 12 (10), 978-988, 2013 | 359 | 2013 |
| Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein PO Bauer, A Goswami, HK Wong, M Okuno, M Kurosawa, M Yamada, ... Nature biotechnology 28 (3), 256-263, 2010 | 291 | 2010 |
| The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies PO Bauer, N Nukina Journal of neurochemistry 110 (6), 1737-1765, 2009 | 255 | 2009 |
| (Patho‐) physiological relevance of PINK 1‐dependent ubiquitin phosphorylation FC Fiesel, M Ando, R Hudec, AR Hill, M Castanedes‐Casey, TR Caulfield, ... EMBO reports 16 (9), 1114-1130, 2015 | 169 | 2015 |
| Blocking acid-sensing ion channel 1 alleviates Huntington's disease pathology via an ubiquitin-proteasome system-dependent mechanism HK Wong, PO Bauer, M Kurosawa, A Goswami, C Washizu, Y Machida, ... Human molecular genetics 17 (20), 3223-3235, 2008 | 153 | 2008 |
| RNA-binding protein TLS is a major nuclear aggregate-interacting protein in huntingtin exon 1 with expanded polyglutamine-expressing cells H Doi, K Okamura, PO Bauer, Y Furukawa, H Shimizu, M Kurosawa, ... Journal of Biological Chemistry 283 (10), 6489-6500, 2008 | 149 | 2008 |
| Inhibition of Rho kinases enhances the degradation of mutant huntingtin PO Bauer, HK Wong, F Oyama, A Goswami, M Okuno, Y Kino, H Miyazaki, ... Journal of Biological Chemistry 284 (19), 13153-13164, 2009 | 130 | 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 | 124 | 2014 |
| Characterization of DNA hypermethylation in the cerebellum of c9FTD/ALS patients VV Belzil, PO Bauer, TF Gendron, ME Murray, D Dickson, L Petrucelli Brain research 1584, 15-21, 2014 | 112 | 2014 |
| Chitin digestibility is dependent on feeding behaviors, which determine acidic chitinase mRNA levels in mammalian and poultry stomachs E Tabata, A Kashimura, A Kikuchi, H Masuda, R Miyahara, Y Hiruma, ... Scientific Reports 8 (1), 1461, 2018 | 85 | 2018 |
| Acidic mammalian chitinase is a proteases-resistant glycosidase in mouse digestive system M Ohno, M Kimura, H Miyazaki, K Okawa, R Onuki, C Nemoto, E Tabata, ... Scientific reports 6 (1), 37756, 2016 | 79 | 2016 |
| Gastric and intestinal proteases resistance of chicken acidic chitinase nominates chitin-containing organisms for alternative whole edible diets for poultry E Tabata, A Kashimura, S Wakita, M Ohno, M Sakaguchi, Y Sugahara, ... Scientific Reports 7 (1), 6662, 2017 | 75 | 2017 |
| Expanded polyglutamines impair synaptic transmission and ubiquitin–proteasome system in Caenorhabditis elegans LA Khan, PO Bauer, H Miyazaki, KS Lindenberg, BG Landwehrmeyer, ... Journal of neurochemistry 98 (2), 576-587, 2006 | 69 | 2006 |
| 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 | 62 | 2016 |
| Epigenetics DNA methylation in the core ataxin-2 gene promoter: novel physiological and pathological implications JM Laffita-Mesa, PO Bauer, V Kourí, L Peña Serrano, J Roskams, ... Human genetics 131, 625-638, 2012 | 60 | 2012 |
| LRRK2 contributes to monocyte dysregulation in Parkinson’s disease C Bliederhaeuser, L Zondler, V Grozdanov, WP Ruf, D Brenner, ... Acta neuropathologica communications 4, 1-5, 2016 | 51 | 2016 |
| Loss and gain of human acidic mammalian chitinase activity by nonsynonymous SNPs K Okawa, M Ohno, A Kashimura, M Kimura, Y Kobayashi, M Sakaguchi, ... Molecular biology and evolution 33 (12), 3183-3193, 2016 | 48 | 2016 |