| The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity B Burnett, F Li, RN Pittman Human molecular genetics 12 (23), 3195-3205, 2003 | 473 | 2003 |
| Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice L Kong, X Wang, DW Choe, M Polley, BG Burnett, M Bosch-Marcé, ... Journal of Neuroscience 29 (3), 842-851, 2009 | 461 | 2009 |
| Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy AM Avila, BG Burnett, AA Taye, F Gabanella, MA Knight, P Hartenstein, ... The Journal of clinical investigation 117 (3), 659-671, 2007 | 434 | 2007 |
| Mutations in TRPV4 cause Charcot-Marie-Tooth disease type 2C G Landouré, AA Zdebik, TL Martinez, BG Burnett, HC Stanescu, H Inada, ... Nature genetics 42 (2), 170-174, 2010 | 356 | 2010 |
| Regulation of SMN protein stability BG Burnett, E Munoz, A Tandon, DY Kwon, CJ Sumner, KH Fischbeck Molecular and cellular biology 29 (5), 1107-1115, 2009 | 356 | 2009 |
| MicroRNA pathways modulate polyglutamine-induced neurodegeneration J Bilen, N Liu, BG Burnett, RN Pittman, NM Bonini Molecular cell 24 (1), 157-163, 2006 | 309 | 2006 |
| The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation BG Burnett, RN Pittman Proceedings of the National Academy of Sciences 102 (12), 4330-4335, 2005 | 227 | 2005 |
| Sustained improvement of spinal muscular atrophy mice treated with trichostatin A plus nutrition HL Narver, L Kong, BG Burnett, DW Choe, M Bosch‐Marcé, AA Taye, ... Annals of neurology 64 (4), 465-470, 2008 | 191 | 2008 |
| Mitochondrial abnormalities in spinal and bulbar muscular atrophy S Ranganathan, GG Harmison, K Meyertholen, M Pennuto, BG Burnett, ... Human molecular genetics 18 (1), 27-42, 2009 | 184 | 2009 |
| Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity I Palazzolo, BG Burnett, JE Young, PL Brenne, AR La Spada, ... Human molecular genetics 16 (13), 1593-1603, 2007 | 161 | 2007 |
| Cowchock syndrome is associated with a mutation in apoptosis-inducing factor C Rinaldi, C Grunseich, IF Sevrioukova, A Schindler, I Horkayne-Szakaly, ... The American Journal of Human Genetics 91 (6), 1095-1102, 2012 | 148 | 2012 |
| Hereditary Spastic Paraplegia Type 43 (SPG43) is Caused by Mutation in C19orf12 G Landouré, PP Zhu, CM Lourenço, JO Johnson, C Toro, KV Bricceno, ... Human mutation 34 (10), 1357-1360, 2013 | 98 | 2013 |
| Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice DY Kwon, WW Motley, KH Fischbeck, BG Burnett Human molecular genetics 20 (18), 3667-3677, 2011 | 93 | 2011 |
| Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics KV Bricceno, T Martinez, E Leikina, S Duguez, TA Partridge, ... Human molecular genetics 23 (18), 4745-4757, 2014 | 81 | 2014 |
| Non-targeted identification of prions and amyloid-forming proteins from yeast and mammalian cells D Kryndushkin, N Pripuzova, BG Burnett, F Shewmaker Journal of Biological Chemistry 288 (38), 27100-27111, 2013 | 73 | 2013 |
| Histone deacetylase inhibition suppresses myogenin-dependent atrogene activation in spinal muscular atrophy mice KV Bricceno, PJ Sampognaro, JP Van Meerbeke, CJ Sumner, ... Human molecular genetics 21 (20), 4448-4459, 2012 | 68 | 2012 |
| The E3 ubiquitin ligase mind bomb 1 ubiquitinates and promotes the degradation of survival of motor neuron protein DY Kwon, M Dimitriadi, B Terzic, C Cable, AC Hart, A Chitnis, ... Molecular biology of the cell 24 (12), 1863-1871, 2013 | 66 | 2013 |
| The role of the immunoproteasome in interferon-γ-mediated microglial activation KE Moritz, NM McCormack, MB Abera, C Viollet, YJ Yauger, G Sukumar, ... Scientific reports 7 (1), 9365, 2017 | 54 | 2017 |
| Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics BG Burnett, J Andrews, S Ranganathan, KH Fischbeck, NA Di Prospero Neurobiology of disease 30 (3), 365-374, 2008 | 51 | 2008 |
| CNS uptake of bortezomib is enhanced by P-glycoprotein inhibition: implications for spinal muscular atrophy E Foran, DY Kwon, JH Nofziger, ES Arnold, MD Hall, KH Fischbeck, ... Neurobiology of disease 88, 118-124, 2016 | 45 | 2016 |
