The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease SE Andrew, Y Paul Goldberg, B Kremer, H Telenius, J Theilmann, ... Nature genetics 4 (4), 398-403, 1993 | 1487 | 1993 |
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease EJ Slow, J van Raamsdonk, D Rogers, SH Coleman, RK Graham, Y Deng, ... Human molecular genetics 12 (13), 1555-1567, 2003 | 945 | 2003 |
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract YP Goldberg, DW Nicholson, DM Rasper, MA Kalchman, HB Koide, ... Nature genetics 13 (4), 442-449, 1996 | 833 | 1996 |
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin RK Graham, Y Deng, EJ Slow, B Haigh, N Bissada, G Lu, J Pearson, ... Cell 125 (6), 1179-1191, 2006 | 813 | 2006 |
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin S Okamoto, MA Pouladi, M Talantova, D Yao, P Xia, DE Ehrnhoefer, ... Nature medicine 15 (12), 1407-1413, 2009 | 640* | 2009 |
Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice AJ Milnerwood, CM Gladding, MA Pouladi, AM Kaufman, RM Hines, ... Neuron 65 (2), 178-190, 2010 | 588 | 2010 |
Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms M Saleh, JP Vaillancourt, RK Graham, M Huyck, SM Srinivasula, ... Nature 429 (6987), 75-79, 2004 | 528 | 2004 |
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease CL Wellington, LM Ellerby, CA Gutekunst, D Rogers, S Warby, ... Journal of Neuroscience 22 (18), 7862-7872, 2002 | 506 | 2002 |
HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain MA Kalchman, HB Koide, K McCutcheon, RK Graham, K Nichol, ... Nature genetics 16 (1), 44-53, 1997 | 471 | 1997 |
Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme MA Kalchman, RK Graham, G Xia, HB Koide, JG Hodgson, KC Graham, ... Journal of Biological Chemistry 271 (32), 19385-19394, 1996 | 470 | 1996 |
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions EJ Slow, RK Graham, AP Osmand, RS Devon, G Lu, Y Deng, J Pearson, ... Proceedings of the National Academy of Sciences 102 (32), 11402-11407, 2005 | 376 | 2005 |
Molecular analysis of new mutations for Huntington's disease: intermediate alleles and sex of origin effects YP Goldberg, B Kremer, SE Andrew, J Theilmann, RK Graham, F Squitieri, ... Nature genetics 5 (2), 174-179, 1993 | 310 | 1993 |
Wild‐type huntingtin protects neurons from excitotoxicity BR Leavitt, JM van Raamsdonk, J Shehadeh, H Fernandes, Z Murphy, ... Journal of neurochemistry 96 (4), 1121-1129, 2006 | 257 | 2006 |
Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice P Guidetti, GP Bates, RK Graham, MR Hayden, BR Leavitt, ... Neurobiology of disease 23 (1), 190-197, 2006 | 187 | 2006 |
Huntington disease NS Caron, GEB Wright, MR Hayden GeneReviews®[Internet], 2020 | 182 | 2020 |
Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease CE Wang, S Tydlacka, AL Orr, SH Yang, RK Graham, MR Hayden, S Li, ... Human molecular genetics 17 (17), 2738-2751, 2008 | 165 | 2008 |
Striatal neuronal apoptosis is preferentially enhanced by NMDA receptor activation in YAC transgenic mouse model of Huntington disease J Shehadeh, HB Fernandes, MMZ Mullins, RK Graham, BR Leavitt, ... Neurobiology of disease 21 (2), 392-403, 2006 | 165 | 2006 |
Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells T Ratovitski, M Gucek, H Jiang, E Chighladze, E Waldron, J D'Ambola, ... Journal of Biological Chemistry 284 (16), 10855-10867, 2009 | 163 | 2009 |
Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus SC Warby, CN Doty, RK Graham, JB Carroll, YZ Yang, RR Singaraja, ... Human molecular genetics 17 (15), 2390-2404, 2008 | 162 | 2008 |
Caspase-6 and neurodegeneration RK Graham, DE Ehrnhoefer, MR Hayden Trends in neurosciences 34 (12), 646-656, 2011 | 147 | 2011 |