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Transgenic rat model of Huntington's disease S von Hörsten, I Schmitt, HP Nguyen, C Holzmann, T Schmidt, T Walther, ... Human molecular genetics 12 (6), 617-624, 2003 | 422 | 2003 |
Mutational spectrum in a worldwide study of 29,700 families with BRCA1 or BRCA2 mutations TR Rebbeck, TM Friebel, E Friedman, U Hamann, D Huo, A Kwong, ... Human mutation 39 (5), 593-620, 2018 | 327 | 2018 |
Neurodegeneration and motor dysfunction in a conditional model of Parkinson's disease S Nuber, E Petrasch-Parwez, B Winner, J Winkler, S von Hörsten, ... Journal of Neuroscience 28 (10), 2471-2484, 2008 | 226 | 2008 |
Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity H Zhou, F Cao, Z Wang, ZX Yu, HP Nguyen, J Evans, SH Li, XJ Li The Journal of cell biology 163 (1), 109-118, 2003 | 188 | 2003 |
Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes M Valenza, V Leoni, JM Karasinska, L Petricca, J Fan, J Carroll, ... Journal of Neuroscience 30 (32), 10844-10850, 2010 | 178 | 2010 |
The regulation of OXPHOS by extramitochondrial calcium FN Gellerich, Z Gizatullina, S Trumbeckaite, HP Nguyen, T Pallas, ... Biochimica et Biophysica Acta (BBA)-Bioenergetics 1797 (6-7), 1018-1027, 2010 | 159 | 2010 |
Lack of huntingtin-associated protein-1 causes neuronal death resembling hypothalamic degeneration in Huntington's disease SH Li, ZX Yu, CL Li, HP Nguyen, YX Zhou, C Deng, XJ Li Journal of Neuroscience 23 (17), 6956-6964, 2003 | 158 | 2003 |
Overexpression of human wildtype torsinA and human ΔGAG torsinA in a transgenic mouse model causes phenotypic abnormalities K Grundmann, B Reischmann, G Vanhoutte, J Hübener, P Teismann, ... Neurobiology of disease 27 (2), 190-206, 2007 | 152 | 2007 |
Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice ZX Yu, SH Li, HP Nguyen, XJ Li Human molecular genetics 11 (8), 905-914, 2002 | 147 | 2002 |
Sex differences in a transgenic rat model of Huntington's disease: decreased 17β-estradiol levels correlate with reduced numbers of DARPP32+ neurons in males FJ Bode, M Stephan, H Suhling, R Pabst, RH Straub, KA Raber, M Bonin, ... Human molecular genetics 17 (17), 2595-2609, 2008 | 140 | 2008 |
Behavioral abnormalities precede neuropathological markers in rats transgenic for Huntington's disease HP Nguyen, P Kobbe, H Rahne, T Wörpel, B Jäger, M Stephan, R Pabst, ... Human molecular genetics 15 (21), 3177-3194, 2006 | 137 | 2006 |
Germline antibody recognition of distinct carbohydrate epitopes HP Nguyen, NOL Seto, CR MacKenzie, L Brade, P Kosma, H Brade, ... Nature Structural & Molecular Biology 10 (12), 1019-1025, 2003 | 137 | 2003 |
Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3) J Hübener, JJ Weber, C Richter, L Honold, A Weiss, F Murad, P Breuer, ... Human molecular genetics 22 (3), 508-518, 2013 | 121 | 2013 |
A novel BACHD transgenic rat exhibits characteristic neuropathological features of Huntington disease L Yu-Taeger, E Petrasch-Parwez, AP Osmand, A Redensek, S Metzger, ... Journal of Neuroscience 32 (44), 15426-15438, 2012 | 121 | 2012 |
Stem cell quiescence in the hippocampal neurogenic niche is associated with elevated transforming growth factor-β signaling in an animal model of Huntington disease M Kandasamy, S Couillard-Despres, KA Raber, M Stephan, B Lehner, ... Journal of Neuropathology & Experimental Neurology 69 (7), 717-728, 2010 | 117 | 2010 |
Age at onset in Huntington’s disease is modified by the autophagy pathway: implication of the V471A polymorphism in Atg7 S Metzger, M Saukko, H Van Che, L Tong, Y Puder, O Riess, HP Nguyen Human genetics 128, 453-459, 2010 | 116 | 2010 |
Mitochondria and energetic depression in cell pathophysiology E Seppet, M Gruno, A Peetsalu, Z Gizatullina, HP Nguyen, S Vielhaber, ... International journal of molecular sciences 10 (5), 2252-2303, 2009 | 112 | 2009 |
Huntingtin-associated protein-1 is a modifier of the age-at-onset of Huntington's disease S Metzger, J Rong, HP Nguyen, A Cape, J Tomiuk, AS Soehn, P Propping, ... Human molecular genetics 17 (8), 1137-1146, 2008 | 112 | 2008 |
Cellular and subcellular localization of Huntington aggregates in the brain of a rat transgenic for Huntington disease E Petrasch‐Parwez, HP Nguyen, M Löbbecke‐Schumacher, HW Habbes, ... Journal of Comparative Neurology 501 (5), 716-730, 2007 | 110 | 2007 |