| Polyglutamine spinocerebellar ataxias—from genes to potential treatments HL Paulson, VG Shakkottai, HB Clark, HT Orr Nature Reviews Neuroscience 18 (10), 613-626, 2017 | 363 | 2017 |
| Genome sequencing analysis identifies new loci associated with Lewy body dementia and provides insights into its genetic architecture R Chia, MS Sabir, S Bandres-Ciga, S Saez-Atienzar, RH Reynolds, ... Nature genetics 53 (3), 294-303, 2021 | 277 | 2021 |
| Polyglutamine repeats in neurodegenerative diseases AP Lieberman, VG Shakkottai, RL Albin Annual Review of Pathology: Mechanisms of Disease 14 (1), 1-27, 2019 | 274 | 2019 |
| Modulators of small-and intermediate-conductance calcium-activated potassium channels and their therapeutic indications H Wulff, A Kolski-Andreaco, A Sankaranarayanan, JM Sabatier, ... Current medicinal chemistry 14 (13), 1437-1457, 2007 | 267 | 2007 |
| Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3 VG Shakkottai, M do Carmo Costa, JM Dell'Orco, A Sankaranarayanan, ... Journal of Neuroscience 31 (36), 13002-13014, 2011 | 238 | 2011 |
| Current opinions and areas of consensus on the role of the cerebellum in dystonia VG Shakkottai, A Batla, K Bhatia, WT Dauer, C Dresel, M Niethammer, ... The Cerebellum 16, 577-594, 2017 | 228 | 2017 |
| Mutations in KCND3 cause spinocerebellar ataxia type 22 YC Lee, A Durr, K Majczenko, YH Huang, YC Liu, CC Lien, PC Tsai, ... Annals of neurology 72 (6), 859-869, 2012 | 185 | 2012 |
| Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration MJ Elrick, CD Pacheco, T Yu, N Dadgar, VG Shakkottai, C Ware, ... Human molecular genetics 19 (5), 837-847, 2010 | 166 | 2010 |
| Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study T Ashizawa, KP Figueroa, SL Perlman, CM Gomez, GR Wilmot, ... Orphanet journal of rare diseases 8, 1-8, 2013 | 162 | 2013 |
| Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice HS McLoughlin, LR Moore, R Chopra, R Komlo, M McKenzie, ... Annals of neurology 84 (1), 64-77, 2018 | 155 | 2018 |
| Mutations in VPS13D lead to a new recessive ataxia with spasticity and mitochondrial defects E Seong, R Insolera, M Dulovic, EJ Kamsteeg, J Trinh, N Brüggemann, ... Annals of neurology 83 (6), 1075-1088, 2018 | 152 | 2018 |
| SKCa channels mediate the medium but not the slow calcium-activated afterhyperpolarization in cortical neurons C Villalobos, VG Shakkottai, KG Chandy, SK Michelhaugh, R Andrade Journal of Neuroscience 24 (14), 3537-3542, 2004 | 149 | 2004 |
| FGF14 regulates the intrinsic excitability of cerebellar Purkinje neurons VG Shakkottai, M Xiao, L Xu, M Wong, JM Nerbonne, DM Ornitz, ... Neurobiology of disease 33 (1), 81-88, 2009 | 138 | 2009 |
| Enhanced neuronal excitability in the absence of neurodegeneration induces cerebellar ataxia VG Shakkottai, C Chou, S Oddo, CA Sailer, HG Knaus, GA Gutman, ... The Journal of clinical investigation 113 (4), 582-590, 2004 | 130 | 2004 |
| Design and characterization of a highly selective peptide inhibitor of the small conductance calcium-activated K+ channel, SkCa2 VG Shakkottai, I Regaya, H Wulff, Z Fajloun, H Tomita, M Fathallah, ... Journal of Biological Chemistry 276 (46), 43145-43151, 2001 | 130 | 2001 |
| Clinical neurogenetics: autosomal dominant spinocerebellar ataxia VG Shakkottai, BL Fogel Neurologic clinics 31 (4), 987-1007, 2013 | 125 | 2013 |
| Neuronal atrophy early in degenerative ataxia is a compensatory mechanism to regulate membrane excitability JM Dell'Orco, AH Wasserman, R Chopra, MAC Ingram, YS Hu, V Singh, ... Journal of Neuroscience 35 (32), 11292-11307, 2015 | 122 | 2015 |
| Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD ML Schultz, KL Krus, S Kaushik, D Dang, R Chopra, L Qi, VG Shakkottai, ... Nature communications 9 (1), 3671, 2018 | 117 | 2018 |
| Toward RNAi therapy for the polyglutamine disease Machado–Joseph disease M do Carmo Costa, K Luna-Cancalon, S Fischer, NS Ashraf, M Ouyang, ... Molecular Therapy 21 (10), 1898-1908, 2013 | 116 | 2013 |
| Depression and clinical progression in spinocerebellar ataxias RY Lo, KP Figueroa, SM Pulst, S Perlman, G Wilmot, C Gomez, ... Parkinsonism & related disorders 22, 87-92, 2016 | 115 | 2016 |