Potassium channel dysfunction underlies Purkinje neuron spiking abnormalities in spinocerebellar ataxia type 2

Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method

Livak, K. J., & Schmittgen, T. D. (2001) · Methods · doi:10.1006/meth.2001.1262 · Cited by 180,860 · see citing works

Mutation of the mouse klotho gene leads to a syndrome resembling ageing

Kuro-o, M., Matsumura, Y., Aizawa, H., Kawaguchi, H., Suga, T., Utsugi, T., Ohyama, Y., Kurabayashi, M., Kaname, T., Kume, E., et al. (1997) · Nature · doi:10.1038/36285 · Cited by 3,837 · see citing works

Inwardly Rectifying Potassium Channels: Their Structure, Function, and Physiological Roles

Hibino, H., Inanobe, A., Furutani, K., Murakami, S., Findlay, I., & Kurachi, Y. (2010) · Physiological Reviews · doi:10.1152/physrev.00021.2009 · Cited by 1,518 · see citing works

Tonic Synaptic Inhibition Modulates Neuronal Output Pattern and Spatiotemporal Synaptic Integration

Häusser, M., & Clark, B. A. (1997) · Neuron · doi:10.1016/s0896-6273(00)80379-7 · Cited by 638 · see citing works

Discharge of Purkinje and cerebellar nuclear neurons during rapidly alternating arm movements in the monkey.

Thach, W. T. (1968) · Journal of Neurophysiology · doi:10.1152/jn.1968.31.5.785 · Cited by 503 · see citing works

Correlation of neural discharge with pattern and force of muscular activity, joint position, and direction of intended next movement in motor cortex and cerebellum

Thach, W. T. (1978) · Journal of Neurophysiology · doi:10.1152/jn.1978.41.3.654 · Cited by 482 · see citing works

Ionic Currents Underlying Spontaneous Action Potentials in Isolated Cerebellar Purkinje Neurons

Raman, I. M., & Bean, B. P. (1999) · Journal of Neuroscience · doi:10.1523/jneurosci.19-05-01663.1999 · Cited by 456 · see citing works

Decreases in the precision of Purkinje cell pacemaking cause cerebellar dysfunction and ataxia

Walter, J. T., Alviña, K., Womack, M. D., Chevez, C., & Khodakhah, K. (2006) · Nature Neuroscience · doi:10.1038/nn1648 · Cited by 435 · see citing works

Cerebellar ataxia and Purkinje cell dysfunction caused by Ca 2+ -activated K + channel deficiency

Sausbier, M., Hu, H., Arntz, C., Feil, S. C., Kamm, S., Adelsberger, H., Sausbier, U., Sailer, C. A., Feil, R., Hofmann, F. B., et al. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0401702101 · Cited by 414 · see citing works

Spatiotemporal firing patterns in the cerebellum

Zeeuw, C. I. D., Hoebeek, F. E., Bosman, L. W. J., Schonewille, M., Witter, L., & Koekkoek, S. K. E. (2011) · Nature reviews. Neuroscience · doi:10.1038/nrn3011 · Cited by 411 · see citing works

Mutations in voltage-gated potassium channel KCNC3 cause degenerative and developmental central nervous system phenotypes

Waters, M. F., Minassian, N. A., Stévanin, G., Figueroa, K. P., Bannister, J. P., Nolte, D., Mock, A. F., Evidente, V. G. H., Fee, D. B., Müller, U., et al. (2006) · Nature Genetics · doi:10.1038/ng1758 · Cited by 312 · see citing works

Discharge properties of Purkinje cells recorded on single and double microelectrodes.

Bell, C. C., & Grimm, R. J. (1969) · Journal of Neurophysiology · doi:10.1152/jn.1969.32.6.1044 · Cited by 280 · see citing works

Inward rectifier K+ currents in smooth muscle cells from rat resistance-sized cerebral arteries

Quayle, J. M., McCarron, J. G., Brayden, J. E., & Nelson, M. T. (1993) · American Journal of Physiology-Cell Physiology · doi:10.1152/ajpcell.1993.265.5.c1363 · Cited by 257 · see citing works

Distinct contributions of small and large conductance Ca2+-activated K+ channels to rat Purkinje neuron function

Edgerton, J. R., & Reinhart, P. H. (2003) · The Journal of Physiology · doi:10.1111/j.1469-7793.2003.00053.x · Cited by 237 · see citing works

Early Changes in Cerebellar Physiology Accompany Motor Dysfunction in the Polyglutamine Disease Spinocerebellar Ataxia Type 3

Shakkottai, V. G., Costa, M. D. C., Dell’Orco, J., Sankaranarayanan, A., Wulff, H., & Paulson, H. L. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.2789-11.2011 · Cited by 215 · see citing works

Distinct contributions of small and large conductance Ca2+-activated K+ channels to rat Purkinje neuron function

Edgerton, J. R., & Reinhart, P. H. (2003) · The Journal of Physiology · doi:10.1113/jphysiol.2002.027854 · Cited by 197 · see citing works

Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2

Hansen, S. T., Meera, P., Otis, T. S., & Pulst, S. M. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds427 · Cited by 176 · see citing works

Aminopyridines Correct Early Dysfunction and Delay Neurodegeneration in a Mouse Model of Spinocerebellar Ataxia Type 1

Hourez, R., Servais, L., Orduz, D., Gall, D., Millard, I., d’Exaerde, A. D. K., Cheron, G. A., Orr, H. T., Pandolfo, M., & Schiffmann, S. N. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.0905-11.2011 · Cited by 153 · see citing works

Selective Positive Modulator of Calcium-Activated Potassium Channels Exerts Beneficial Effects in a Mouse Model of Spinocerebellar Ataxia Type 2

Kasumu, A. W., Hougaard, C. Ø., Rode, F., Jacobsen, T. A., Sabatier, J., Eriksen, B. L., Strøbæk, D., Liang, X., Egorova, P. A., Vorontsova, D., et al. (2012) · Chemistry & Biology · doi:10.1016/j.chembiol.2012.07.013 · Cited by 148 · see citing works

Developmental Regulation of Small-Conductance Ca2+-Activated K+ Channel Expression and Function in Rat Purkinje Neurons

Cingolani, L. A., Gymnopoulos, M., Boccaccio, A., Stocker, M., & Pedarzani, P. (2002) · Journal of Neuroscience · doi:10.1523/jneurosci.22-11-04456.2002 · Cited by 145 · see citing works

Loss of β-III Spectrin Leads to Purkinje Cell Dysfunction Recapitulating the Behavior and Neuropathology of Spinocerebellar Ataxia Type 5 in Humans

Perkins, E., Clarkson, Y. L., Sabatier, N., Longhurst, D. M., Millward, C. P., Jack, J., Toraiwa, J., Watanabe, M., Rothstein, J. D., Lyndon, A. R., et al. (2010) · Journal of Neuroscience · doi:10.1523/jneurosci.6065-09.2010 · Cited by 134 · see citing works

FGF14 Regulates the Intrinsic Excitability of Cerebellar Purkinje Neurons

Shakkottai, V., Xiao, M.-L., Xu, L., Wong, M., Nerbonne, J., Ornitz, D., & Yamada, K. A. (2008) · Neurobiology of Disease · doi:10.1016/j.nbd.2008.09.019 · Cited by 130 · see citing works

Interaction of Kv3 Potassium Channels and Resurgent Sodium Current Influences the Rate of Spontaneous Firing of Purkinje Neurons

Akemann, W., & Knöpfel, T. (2006) · Journal of Neuroscience · doi:10.1523/jneurosci.5204-05.2006 · Cited by 129 · see citing works

SKCaChannels Mediate the Medium But Not the Slow Calcium-Activated Afterhyperpolarization in Cortical Neurons

Villalobos, C. A., Shakkottai, V. G., Chandy, K. G., Michelhaugh, S. K., & Andrade, R. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.0380-04.2004 · Cited by 126 · see citing works

Enhanced neuronal excitability in the absence of neurodegeneration induces cerebellar ataxia

Shakkottai, V. G., Chou, C.-H., Oddo, S., Sailer, C. A., Knaus, H., Gutman, G. A., Barish, M. E., LaFerla, F. M., & Chandy, K. G. (2004) · Journal of Clinical Investigation · doi:10.1172/jci200420216 · Cited by 117 · see citing works

Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability

Dell’Orco, J., Wasserman, A. H., Chopra, R., Ingram, M. A. C., Hu, Y., Singh, V., Wulff, H., Opal, P., Orr, H. T., & Shakkottai, V. G. (2015) · Journal of Neuroscience · doi:10.1523/jneurosci.1357-15.2015 · Cited by 109 · see citing works

Molecular Aspects of Structure, Gating, and Physiology of pH-Sensitive Background K 2P and Kir K + -Transport Channels

Sepúlveda, F. V., Cid, L. P., Teulon, J., & Niemeyer, M. I. (2014) · Physiological Reviews · doi:10.1152/physrev.00016.2014 · Cited by 105 · see citing works

Mechanism of Ba2+ block of a mouse inwardly rectifying K+ channel: differential contribution by two discrete residues

Alagem, N., Dvir, M., & Reuveny, E. (2001) · The Journal of Physiology · doi:10.1111/j.1469-7793.2001.00381.x · Cited by 104 · see citing works

Disruption of the olivo-cerebellar circuit by Purkinje neuron-specific ablation of BK channels

Chen, X., Kovalchuk, Y., Adelsberger, H., Henning, H. A., Sausbier, M., Wietzorrek, G., Ruth, P., Yarom, Y., & Konnerth, A. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1001745107 · Cited by 98 · see citing works

Ataxin-2 Regulates RGS8 Translation in a New BAC-SCA2 Transgenic Mouse Model

Dansithong, W., Paul, S., Figueroa, K. P., Rinehart, M. D., Wiest, S., Pflieger, L. T., Scoles, D. R., & Pulst, S. M. (2015) · PLoS Genetics · doi:10.1371/journal.pgen.1005182 · Cited by 95 · see citing works

Clinical Neurogenetics

Shakkottai, V. G., & Fogel, B. L. (2013) · Neurologic Clinics · doi:10.1016/j.ncl.2013.04.006 · Cited by 81 · see citing works

Loss of beta-III spectrin leads to purkinje cell dysfunction recapitulating the behavior and neuropathology of soinocerebellar ataxia type 5 in humans

Perkins, E., Clarkson, Y. L., Sabatier, N., Longhurst, D. M., Millward, C. P., Jack, J. L., Toraiwa, J., Watanabi, M., Rothstein, J. D., Lyndon, A. R., et al. (2010) · Journal of Neuroscience Research · Cited by 74 · see citing works

External Ba2+ Block of the Two-pore Domain Potassium Channel TREK-1 Defines Conformational Transition in Its Selectivity Filter

Ma, X., Yu, J.-M., Zhang, S., Liu, X., Wu, B., Wei, X.-L., Yan, J., Hong-liang, S., Yan, H., & Zheng, J. (2011) · Journal of Biological Chemistry · doi:10.1074/jbc.m111.264788 · Cited by 51 · see citing works

Gene co-expression network analysis for identifying modules and functionally enriched pathways in SCA2

Pflieger, L. T., Dansithong, W., Paul, S., Scoles, D. R., Figueroa, K. P., Meera, P., Otis, T. S., Facelli, J. C., & Pulst, S. M. (2017) · Human Molecular Genetics · doi:10.1093/hmg/ddx191 · Cited by 51 · see citing works

Enhanced neuronal excitability in the absence of neurodegeneration induces cerebellar ataxia

Shakkottai, V. G., Chou, C.-H., Oddo, S., et al. (2004) · Journal of Clinical Investigation · doi:10.1172/jci20216 · Cited by 48 · see citing works

Iberiotoxin-sensitive and -insensitive BK currents in Purkinje neuron somata

Benton, M. D., Lewis, A. H., Bant, J. S., & Raman, I. M. (2013) · Journal of Neurophysiology · doi:10.1152/jn.00127.2012 · Cited by 47 · see citing works

Inwardly rectifying K+ channels influence Ca2+ entry due to nucleotide receptor activation in microglia

Franchini, L., Levi, G., & Visentin, S. (2004) · Cell Calcium · doi:10.1016/j.ceca.2003.11.001 · Cited by 44 · see citing works

Pharmacological characterization of a non‐inactivating outward current observed in mouse cerebellar Purkinje neurones

Bushell, T. J., Clarke, C. E., Mathie, A. A., & Robertson, B. (2002) · British Journal of Pharmacology · doi:10.1038/sj.bjp.0704518 · Cited by 43 · see citing works

Precision medicine in spinocerebellar ataxias: treatment based on common mechanisms of disease.

Bushart, D. D., Murphy, G. G., & Shakkottai, V. G. (2016) · PubMed Central · doi:10.3978/j.issn.2305-5839.2016.01.06 · Cited by 36 · see citing works

Translating Cerebellar Purkinje Neuron Physiology To Progress In Dominantly Inherited Ataxia

Chopra, R., & Shakkottai, V. G. (2014) · Future Neurology · doi:10.2217/fnl.14.6 · Cited by 30 · see citing works

Inward-Rectifying Potassium (K ir ) Channels Regulate Pacemaker Activity in Spinal Nociceptive Circuits during Early Life

Li, J., Blankenship, M. L., & Baccei, M. L. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.4365-12.2013 · Cited by 30 · see citing works

The Role for Alterations in Neuronal Activity in the Pathogenesis of Polyglutamine Repeat Disorders

Chopra, R., & Shakkottai, V. (2014) · Neurotherapeutics · doi:10.1007/s13311-014-0289-7 · Cited by 22 · see citing works

Selectivity and interactions of Ba2+ and Cs+ with wild‐type and mutant TASK1 K+ channels expressed in Xenopus oocytes

O'Connell, A. D., Morton, M. J., Sivaprasadarao, A., & Hunter, M. (2004) · The Journal of Physiology · doi:10.1113/jphysiol.2004.079020 · Cited by 17 · see citing works

Spinocerebellar ataxia

Klockgether, T., Mariotti, C., & Paulson, H. L. (2019) · Nature Reviews Disease Primers · doi:10.1038/s41572-019-0074-3 · Cited by 714 · see citing works

Polyglutamine spinocerebellar ataxias — from genes to potential treatments

Paulson, H. L., Shakkottai, V. G., Clark, H. B., & Orr, H. T. (2017) · Nature reviews. Neuroscience · doi:10.1038/nrn.2017.92 · Cited by 376 · see citing works

Emerging connections between cerebellar development, behaviour and complex brain disorders

Sathyanesan, A., Zhou, J., Scafidi, J., Heck, D. H., Sillitoe, R. V., & Gallo, V. (2019) · Nature reviews. Neuroscience · doi:10.1038/s41583-019-0152-2 · Cited by 322 · see citing works

Polyglutamine Repeats in Neurodegenerative Diseases

Lieberman, A. P., Shakkottai, V. G., & Albin, R. L. (2018) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-012418-012857 · Cited by 299 · see citing works

Spinocerebellar ataxias: prospects and challenges for therapy development

Ashizawa, T., Öz, G., & Paulson, H. L. (2018) · Nature Reviews Neurology · doi:10.1038/s41582-018-0051-6 · Cited by 251 · see citing works

Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice

McLoughlin, H. S., Moore, L. R., Chopra, R., Komlo, R., McKenzie, M., Blumenstein, K. G., Zhao, H. T., Kordasiewicz, H. B., Shakkottai, V. G., & Paulson, H. L. (2018) · Annals of Neurology · doi:10.1002/ana.25264 · Cited by 140 · see citing works

Chronic unpredictable stress induces depression-related behaviors by suppressing AgRP neuron activity

Fang, X., Jiang, S., Wang, J., Bai, Y., Kim, C. S., Blake, D., Weintraub, N. L., Lei, Y., & Lu, X.-Y. (2021) · Molecular Psychiatry · doi:10.1038/s41380-020-01004-x · Cited by 95 · see citing works

Losing the Beat: Contribution of Purkinje Cell Firing Dysfunction to Disease, and Its Reversal

Cook, A. A., Fields, E., & Watt, A. J. (2020) · Neuroscience · doi:10.1016/j.neuroscience.2020.06.008 · Cited by 90 · see citing works

Polyglutamine diseases

Bunting, E. L., Hamilton, J., & Tabrizi, S. J. (2021) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2021.07.001 · Cited by 88 · see citing works

Spinocerebellar Ataxia Type 2

Scoles, D. R., & Pulst, S. M. (2018) · Advances in experimental medicine and biology · doi:10.1007/978-3-319-71779-1_8 · Cited by 85 · see citing works

Ion channel dysfunction in cerebellar ataxia

Bushart, D. D., & Shakkottai, V. G. (2018) · Neuroscience Letters · doi:10.1016/j.neulet.2018.02.005 · Cited by 71 · see citing works

Nicotinamide Pathway-Dependent Sirt1 Activation Restores Calcium Homeostasis to Achieve Neuroprotection in Spinocerebellar Ataxia Type 7

Stoyas, C. A., Bushart, D. D., Switonski, P. M., Ward, J. M., Alaghatta, A., Tang, M., Niu, C., Wadhwa, M., Huang, H., Savchenko, A., et al. (2019) · Neuron · doi:10.1016/j.neuron.2019.11.019 · Cited by 67 · see citing works

Aberrant Cerebellar Circuitry in the Spinocerebellar Ataxias

Robinson, K. J., Watchon, M., & Laird, A. S. (2020) · Frontiers in Neuroscience · doi:10.3389/fnins.2020.00707 · Cited by 61 · see citing works

Targeting potassium channels to treat cerebellar ataxia

Bushart, D. D., Chopra, R., Singh, V., Murphy, G. G., Wulff, H., & Shakkottai, V. G. (2018) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.527 · Cited by 58 · see citing works

Molecular Mechanisms and Therapeutics for Spinocerebellar Ataxia Type 2

Egorova, P. A., & Борисович, Б. И. (2019) · Neurotherapeutics · doi:10.1007/s13311-019-00777-6 · Cited by 57 · see citing works

Understanding the molecular diversity of synapses

van Oostrum, M., & Schuman, E. M. (2025) · Nature Reviews Neuroscience · doi:10.1038/s41583-024-00888-w · Cited by 49 · see citing works

NLRP3 inflammasome inhibition attenuates subacute neurotoxicity induced by acrylamide in vitro and in vivo

Sui, X. Y., Yang, J., Zhang, G., Yuan, X., Li, W.-H., Long, J., Luo, Y., Li, Y., & Wang, Y. (2020) · Toxicology · doi:10.1016/j.tox.2020.152392 · Cited by 46 · see citing works

Generation of an Atxn2-CAG100 knock-in mouse reveals N-acetylaspartate production deficit due to early Nat8l dysregulation

Şen, N.-E., Canet-Pons, J., Halbach, M. V., Arsović, A., Pilatus, U., Chae, W. H., Güleç, Z. E. K., Seidel, K., Rollmann, E., Mittelbronn, M. G. A., et al. (2019) · Neurobiology of Disease · doi:10.1016/j.nbd.2019.104559 · Cited by 44 · see citing works

A Chlorzoxazone‐Baclofen Combination Improves Cerebellar Impairment in Spinocerebellar Ataxia Type 1

Bushart, D. D., Huang, H., Man, L. J., Morrison, L. M., & Shakkottai, V. G. (2020) · Movement Disorders · doi:10.1002/mds.28355 · Cited by 40 · see citing works

Protein kinase C activity is a protective modifier of Purkinje neuron degeneration in cerebellar ataxia

Chopra, R., Wasserman, A. H., Pulst, S. M., Zeeuw, C. I. D., & Shakkottai, V. G. (2018) · Human Molecular Genetics · doi:10.1093/hmg/ddy050 · Cited by 36 · see citing works

A Dynamic Splicing Program Ensures Proper Synaptic Connections in the Developing Cerebellum

Farini, D., Cesari, E., Weatheritt, R. J., Sala, G. L., Naro, C., Pagliarini, V., Bonvissuto, D., Medici, V., Guerra, M., Pietro, C. D., et al. (2020) · Cell Reports · doi:10.1016/j.celrep.2020.107703 · Cited by 32 · see citing works

Potassium and calcium channels in different nerve cells act as therapeutic targets in neurological disorders

Qiu, Q., Yang, M., Gong, D., Liang, H., & Chen, T. (2024) · Neural Regeneration Research · doi:10.4103/nrr.nrr-d-23-01766 · Cited by 33 · see citing works

Acute Cerebellar Inflammation and Related Ataxia: Mechanisms and Pathophysiology

Parvez, M. S. A., & Ohtsuki, G. (2022) · Brain Sciences · doi:10.3390/brainsci12030367 · Cited by 34 · see citing works

Viewpoint: spinocerebellar ataxias as diseases of Purkinje cell dysfunction rather than Purkinje cell loss

Kapfhammer, J. P., & Shimobayashi, E. (2023) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2023.1182431 · Cited by 29 · see citing works

A novel, ataxic mouse model of ataxia telangiectasia caused by a clinically relevant nonsense mutation

Perez, H. D., Abdallah, M. F., Chavira, J. I., Norris, A. S., Egeland, M. T., Vo, K. L., Buechsenschuetz, C. L., Sanghez, V., Kim, J. L., Pind, M., et al. (2021) · eLife · doi:10.7554/elife.64695 · Cited by 27 · see citing works

Antisense Oligonucleotide Therapy Targeted Against ATXN3 Improves Potassium Channel–Mediated Purkinje Neuron Dysfunction in Spinocerebellar Ataxia Type 3

Bushart, D. D., Zalon, A. J., Zhang, H., Morrison, L. M., Guan, Y., Paulson, H. L., Shakkottai, V. G., & McLoughlin, H. S. (2020) · The Cerebellum · doi:10.1007/s12311-020-01179-7 · Cited by 26 · see citing works

Dendritic potassium channel dysfunction may contribute to dendrite degeneration in spinocerebellar ataxia type 1

Chopra, R., Bushart, D. D., & Shakkottai, V. G. (2018) · PLoS ONE · doi:10.1371/journal.pone.0198040 · Cited by 25 · see citing works

Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1

Chopra, R., Bushart, D. D., Cooper, J.-P. R., Yellajoshyula, D., Morrison, L. M., Huang, H.-R., Handler, H. P., Man, L.-K. J., Dansithong, W., Scoles, D., et al. (2020) · bioRxiv · doi:10.1093/hmg/ddaa212 · Cited by 27 · see citing works

Spinocerebellar Ataxia Type 2: A Review and Personal Perspective.

Pulst, S. M. (2025) · PubMed Central · doi:10.1212/nxg.0000000000200225 · Cited by 19 · see citing works

Circuit-Specific Deep Brain Stimulation Provides Insights into Movement Control

Gittis, A. H., & Sillitoe, R. V. (2024) · Annual Review of Neuroscience · doi:10.1146/annurev-neuro-092823-104810 · Cited by 18 · see citing works

Moving Towards Therapy in SCA1: Insights from Molecular Mechanisms, Identification of Novel Targets, and Planning for Human Trials

Srinivasan, S. R., & Shakkottai, V. G. (2019) · Neurotherapeutics · doi:10.1007/s13311-019-00763-y · Cited by 17 · see citing works

ClC-2-like Chloride Current Alterations in a Cell Model of Spinal and Bulbar Muscular Atrophy, a Polyglutamine Disease

Martínez-Rojas, V. A., Jiménez-Garduño, A. M., Michelatti, D., Tosatto, L., Marchioretto, M., Arosio, D., Basso, M., Pennuto, M., & Musio, C. (2020) · Journal of Molecular Neuroscience · doi:10.1007/s12031-020-01687-5 · Cited by 17 · see citing works

Electrophysiological Studies Support Utility of Positive Modulators of SK Channels for the Treatment of Spinocerebellar Ataxia Type 2

Egorova, P. A., & Борисович, Б. И. (2022) · The Cerebellum · doi:10.1007/s12311-021-01349-1 · Cited by 16 · see citing works

Targeting Ion Channels and Purkinje Neuron Intrinsic Membrane Excitability as a Therapeutic Strategy for Cerebellar Ataxia

Huang, H., & Shakkottai, V. G. (2023) · Life · doi:10.3390/life13061350 · Cited by 15 · see citing works

Discovery of Novel Activators of Large-Conductance Calcium-Activated Potassium Channels for the Treatment of Cerebellar Ataxia

Srinivasan, S. R., Huang, H., Chang, W., Nasburg, J. A., Nguyen, H. M., Strassmaier, T., Wulff, H., & Shakkottai, V. G. (2022) · Molecular Pharmacology · doi:10.1124/molpharm.121.000478 · Cited by 15 · see citing works

Progressive myoclonus epilepsyKCNC1variant causes a developmental dendritopathy

Carpenter, J. C., Männikkö, R., Heffner, C. M., Heneine, J., Castañeda, M. S., Lignani, G., & Schorge, S. (2021) · Epilepsia · doi:10.1111/epi.16867 · Cited by 14 · see citing works

A Xenopus oocyte model system to study action potentials

Corbin-Leftwich, A., Small, H., Robinson, H. H., Villalba‐Galea, C. A., & Boland, L. M. (2018) · The Journal of General Physiology · doi:10.1085/jgp.201812146 · Cited by 14 · see citing works

Deep Brain Stimulation of the Interposed Nucleus Reverses Motor Deficits and Stimulates Production of Anti-inflammatory Cytokines in Ataxia Mice

Kumar, G., Asthana, P., Yung, W., Kwan, K. M., Tin, C., & Ma, C. H. E. (2022) · Molecular Neurobiology · doi:10.1007/s12035-022-02872-w · Cited by 12 · see citing works

Rare KCND3 Loss-of-Function Mutation Associated With the SCA19/22

Li, M., Liu, F., Hao, X., Fan, Y., Li, J., Hu, Z., Shi, J., Fan, L., Zhang, S., Ma, D., et al. (2022) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2022.919199 · Cited by 11 · see citing works

Vulnerability of Human Cerebellar Neurons to Degeneration in Ataxia-Causing Channelopathies

Bushart, D. D., & Shakkottai, V. G. (2022) · Frontiers in Systems Neuroscience · doi:10.3389/fnsys.2022.908569 · Cited by 10 · see citing works

Clenbuterol-sensitive delayed outward potassium currents in a cell model of spinal and bulbar muscular atrophy

Martínez-Rojas, V. A., Arosio, D., Pennuto, M., & Musio, C. (2021) · Pflügers Archiv - European Journal of Physiology · doi:10.1007/s00424-021-02559-6 · Cited by 8 · see citing works

K + ‐independent Kir blockade by external Cs + and Ba 2+

Ouanounou, G. (2022) · Physiological Reports · doi:10.14814/phy2.15200 · Cited by 8 · see citing works

Genetic and clinical landscape of childhood cerebellar hypoplasia and atrophy

Sakamoto, M., Iwama, K., Sasaki, M., Ishiyama, A., Komaki, H., SAITO, T. K., Takeshita, E., Shimizu‐Motohashi, Y., Haginoya, K., Kobayashi, T., et al. (2022) · Genetics in Medicine · doi:10.1016/j.gim.2022.08.007 · Cited by 8 · see citing works

Diagnosis of polyglutamine spinocerebellar ataxias by polymerase chain reaction amplification and Sanger sequencing

Chen, C., Fang, X., & Sun, S.-C. (2018) · Molecular Medicine Reports · doi:10.3892/mmr.2018.9043 · Cited by 7 · see citing works

Spinocerebellar Ataxia Type 2

Pulst, S. M. (2006) · Contemporary clinical neuroscience · doi:10.1007/978-3-030-75817-2_25 · Cited by 5 · see citing works

Repeat expansion disorders

Srinivasan, S. R., de Gusmão, C. M., Korecka, J. A., & Khurana, V. (2022) · Elsevier eBooks · doi:10.1016/b978-0-323-85654-6.00048-4 · Cited by 5 · see citing works

Autonomous STING signaling in Purkinje cells drives neurodegeneration independent of type I interferon

Yang, K., Dunn, M., Torres-Ramirez, G., Dobbs, N., Shakkottai, V. G., & Yan, N. (2025) · Cell Reports · doi:10.1016/j.celrep.2025.116480 · Cited by 4 · see citing works

Knockdown of the Non-canonical Wnt Gene Prickle2 Leads to Cerebellar Purkinje Cell Abnormalities While Cerebellar-Mediated Behaviors Remain Intact

Abbott, P. W., Hardie, J. B., Walsh, K. P., Nessler, A. J., Farley, S. J., Freeman, J. H., Wemmie, J. A., Wendt, L. H., Kim, Y.-C., Sowers, L. P., et al. (2024) · The Cerebellum · doi:10.1007/s12311-023-01648-9 · Cited by 4 · see citing works

Cerebellar Purkinje cell firing reduction contributes to aging-related declining motor coordination in mice

Fields, E., Rogers, B. C., Leung, T. C. S., Huang, A., Kern, M., Kontowicz, N., Dolin, H., & Watt, A. J. (2026) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2525795122 · Cited by 3 · see citing works

Riluzole and spinocerebellar ataxia type 2: the ATRIL trial

Velázquez‐Pérez, L., & Rodríguez-Labrada, R. (2022) · The Lancet Neurology · doi:10.1016/s1474-4422(22)00028-x · Cited by 3 · see citing works

What Physiological Features May Contribute to Purkinje Cell Vulnerability in Cerebellar Ataxias?

Benarroch, E. E. (2025) · Neurology · doi:10.1212/wnl.0000000000213961 · Cited by 2 · see citing works

What Is the Role of Potassium Channels in Ataxia?

Benarroch, E. (2021) · Neurology · doi:10.1212/wnl.0000000000012870 · Cited by 2 · see citing works

Targeting Circuit Abnormalities in Neurodegenerative Disease

Srinivasan, S. R. (2022) · Molecular Pharmacology · doi:10.1124/molpharm.122.000563 · Cited by 2 · see citing works

Enhanced mGluR1 function causes motor deficits and region-specific Purkinje cell dysfunction

Ibrahim, M. F., Boyanova, S. T., Cheng, Y. C., Ligneul, C., Bains, R. S., Johnpulle, T. C., Lerch, J. P., Mann, E. O., Oliver, P. L., & Becker, E. B. E. (2026) · Brain · doi:10.1093/brain/awaf477 · Cited by 1 · see citing works

New Approaches in Studies of the Molecular Pathogenesis of Type 2 Spinocerebellar Ataxia

Egorova, P. A., & Борисович, Б. И. (2020) · Neuroscience and Behavioral Physiology · doi:10.1007/s11055-020-00988-x · Cited by 1 · see citing works

Purkinje Cells

Rossi, T., & Isope, P. (2023) · Springer eBooks · doi:10.1007/978-3-031-15070-8_22 · Cited by 1 · see citing works

Resilience to Endoplasmic Reticulum Stress Mitigates Membrane Hyperexcitability Underlying Late Disease Onset in a Murine Model of SCA6

Huang, H., Charron, T. L., Fu, M., Dunn, M., Jones, D. M., Kumar, P., Ishishita, S., Pool, A., Kulkarni, A., Konopka, G., et al. (2025) · Annals of Neurology · doi:10.1002/ana.78042 · Cited by 1 · see citing works

Atxn2 -CAG100-knock-in affects mouse lifespan and vestibulo-cerebellar function via neural disconnectionPreprint

Halbach, M. V., Şen, N.-E., Canet-Pons, J., Kuppens, B. W., Segers, M., Schonewille, M., Rollmann, E., Seidel, K., Rüb, U., Meierhofer, D., et al. (2018) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/333443

The inherited ataxias

Rosenberg, R. N., Khemani, P., & Shakkottai, V. G. (2020) · Elsevier eBooks · doi:10.1016/b978-0-12-813866-3.00005-9

Faculty Opinions recommendation of Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice.

Dürr, A. (2018) · Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature · doi:10.3410/f.733458894.793552253

Seeking Therapies for Spinocerebellar Ataxia: From Gene Silencing to Systems-Based Approaches

Powers, R., Paulson, H. L., & Srinivasan, S. R. (2023) · Contemporary clinical neuroscience · doi:10.1007/978-3-031-24345-5_6

Spinocerebellar Ataxia Type 7: From Mechanistic Pathways to Therapeutic Opportunities

Switonski, P. M., & Spada, A. R. L. (2023) · Contemporary clinical neuroscience · doi:10.1007/978-3-031-24345-5_17

The inherited ataxias

Rosenberg, R. N., & Shakkottai, V. G. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-19176-3.00007-8

Characterizing molecular and behavioral changes arising from ROMK potassium channel deficiency in the cerebellum

Agrimi, J., Dunphy, A., Zhong, J. C., Zhang, W., Sbaiti, N., Turner, D. R., Bernardele, L., Foster, D. B., O'Rourke, B. P., Paolocci, N., et al. (2026) · Frontiers in Behavioral Neuroscience · doi:10.3389/fnbeh.2025.1681149

Cardiovascular Exercise Drives Neuroprotection in a Mouse Model of Spinocerebellar Ataxia 1 Via Rescue of Aberrant Splicing

Soto, I., Bonomo, M., Nelthrope, B. S., Carr, B. D., Palmer, E., Sierra, M., Ertürk, İ., Peppercorn, D., Cox, E., Vasconcellos, T. K., et al. (2026) · Annals of Neurology · doi:10.1002/ana.78282

Electrophysiological Studies Support Utility of Positive Modulators of SK Channels for the Treatment of Spinocerebellar Ataxia Type 2

Egorova, P. A., & Борисович, Б. И. (2022) · The Cerebellum · doi:10.1007/s12311-021-01349-1 · Cited by 16 · see citing works

Selective Positive Modulator of Calcium-Activated Potassium Channels Exerts Beneficial Effects in a Mouse Model of Spinocerebellar Ataxia Type 2

Kasumu, A. W., Hougaard, C. Ø., Rode, F., Jacobsen, T. A., Sabatier, J., Eriksen, B. L., Strøbæk, D., Liang, X., Egorova, P. A., Vorontsova, D., et al. (2012) · Chemistry & Biology · doi:10.1016/j.chembiol.2012.07.013 · Cited by 148 · see citing works

Targeting potassium channels to treat cerebellar ataxia

Bushart, D. D., Chopra, R., Singh, V., Murphy, G. G., Wulff, H., & Shakkottai, V. G. (2018) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.527 · Cited by 58 · see citing works

What Is the Role of Potassium Channels in Ataxia?

Benarroch, E. (2021) · Neurology · doi:10.1212/wnl.0000000000012870 · Cited by 2 · see citing works

In vivo analysis of cerebellar Purkinje cell activity in SCA2 transgenic mouse model

Egorova, P. A., Zakharova, O. A., Власова, О. Л., & Борисович, Б. И. (2016) · Journal of Neurophysiology · doi:10.1152/jn.00913.2015 · Cited by 64 · see citing works