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Vikram G. Shakkottai, Ph.D. - Publications

Affiliations: 
University of California, Irvine, Irvine, CA 
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53 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2023 Huang H, Shakkottai VG. Targeting Ion Channels and Purkinje Neuron Intrinsic Membrane Excitability as a Therapeutic Strategy for Cerebellar Ataxia. Life (Basel, Switzerland). 13. PMID 37374132 DOI: 10.3390/life13061350  0.348
2022 Bushart DD, Shakkottai VG. Vulnerability of Human Cerebellar Neurons to Degeneration in Ataxia-Causing Channelopathies. Frontiers in Systems Neuroscience. 16: 908569. PMID 35757096 DOI: 10.3389/fnsys.2022.908569  0.343
2022 Srinivasan SR, Huang H, Chang WC, Nasburg JA, Nguyen HM, Strassmaier T, Wulff H, Shakkottai VG. Discovery of novel activators of large-conductance calcium-activated potassium channels for the treatment of cerebellar ataxia. Molecular Pharmacology. PMID 35489717 DOI: 10.1124/molpharm.121.000478  0.566
2020 Chopra R, Bushart DD, Cooper JP, Yellajoshyula D, Morrison LM, Huang H, Handler HP, Man LJ, Dansithong W, Scoles DR, Pulst SM, Orr HT, Shakkottai VG. Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1. Human Molecular Genetics. PMID 32964235 DOI: 10.1093/hmg/ddaa212  0.34
2020 Bushart DD, Zalon AJ, Zhang H, Morrison LM, Guan Y, Paulson HL, Shakkottai VG, McLoughlin HS. Antisense Oligonucleotide Therapy Targeted Against ATXN3 Improves Potassium Channel-Mediated Purkinje Neuron Dysfunction in Spinocerebellar Ataxia Type 3. Cerebellum (London, England). PMID 32789747 DOI: 10.1007/S12311-020-01179-7  0.473
2020 Conway KS, Camelo-Piragua S, Fisher-Hubbard A, Perry WR, Shakkottai VG, Venneti S. Multiple system atrophy pathology is associated with primary Sjögren's syndrome. Jci Insight. 5. PMID 32644976 DOI: 10.1172/Jci.Insight.138619  0.322
2020 Costa MDC, Radzwion M, McLoughlin HS, Ashraf NS, Fischer S, Shakkottai VG, Maciel P, Paulson HL, Öz G. In Vivo Molecular Signatures of Cerebellar Pathology in Spinocerebellar Ataxia Type 3. Movement Disorders : Official Journal of the Movement Disorder Society. PMID 32621646 DOI: 10.1002/Mds.28140  0.356
2020 Yang CY, Lai RY, Amokrane N, Lin CY, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Rosenthal LS, Ying SH, Zesiewicz T, et al. Dysphagia in spinocerebellar ataxias type 1, 2, 3 and 6. Journal of the Neurological Sciences. 415: 116878. PMID 32454319 DOI: 10.1016/J.Jns.2020.116878  0.32
2020 Gan SR, Figueroa KP, Xu HL, Perlman S, Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind MD, Xia G, Subramony SH, et al. The impact of ethnicity on the clinical presentations of spinocerebellar ataxia type 3. Parkinsonism & Related Disorders. 72: 37-43. PMID 32105964 DOI: 10.1016/J.Parkreldis.2020.02.004  0.357
2019 Stoyas CA, Bushart DD, Switonski PM, Ward JM, Alaghatta A, Tang MB, Niu C, Wadhwa M, Huang H, Savchenko A, Gariani K, Xie F, Delaney JR, Gaasterland T, Auwerx J, ... Shakkottai VG, et al. Nicotinamide Pathway-Dependent Sirt1 Activation Restores Calcium Homeostasis to Achieve Neuroprotection in Spinocerebellar Ataxia Type 7. Neuron. PMID 31859031 DOI: 10.1016/J.Neuron.2019.11.019  0.37
2019 Moore LR, Keller L, Bushart DD, Delatorre RG, Li D, McLoughlin HS, do Carmo Costa M, Shakkottai VG, Smith GD, Paulson HL. Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line. Stem Cell Research. 39: 101504. PMID 31374463 DOI: 10.1016/J.Scr.2019.101504  0.352
2019 Srinivasan SR, Shakkottai VG. Moving Towards Therapy in SCA1: Insights from Molecular Mechanisms, Identification of Novel Targets, and Planning for Human Trials. Neurotherapeutics : the Journal of the American Society For Experimental Neurotherapeutics. PMID 31338702 DOI: 10.1007/S13311-019-00763-Y  0.365
2019 Lai RY, Tomishon D, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind M, Xia G, et al. Tremor in the Degenerative Cerebellum: Towards the Understanding of Brain Circuitry for Tremor. Cerebellum (London, England). PMID 30830673 DOI: 10.1007/S12311-019-01016-6  0.355
2018 Brown AS, Meera P, Altindag B, Chopra R, Perkins EM, Paul S, Scoles DR, Tarapore E, Magri J, Huang H, Jackson M, Shakkottai VG, Otis TS, Pulst SM, Atwood SX, et al. MTSS1/Src family kinase dysregulation underlies multiple inherited ataxias. Proceedings of the National Academy of Sciences of the United States of America. PMID 30530649 DOI: 10.1073/Pnas.1816177115  0.412
2018 Schultz ML, Krus KL, Kaushik S, Dang D, Chopra R, Qi L, Shakkottai VG, Cuervo AM, Lieberman AP. Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD. Nature Communications. 9: 3671. PMID 30202070 DOI: 10.1038/S41467-018-06115-2  0.318
2018 Lieberman AP, Shakkottai VG, Albin RL. Polyglutamine Repeats in Neurodegenerative Diseases. Annual Review of Pathology. PMID 30089230 DOI: 10.1146/Annurev-Pathmechdis-012418-012857  0.37
2018 Sun M, Johnson AK, Nelakuditi V, Guidugli L, Fischer D, Arndt K, Ma L, Sandford E, Shakkottai V, Boycott K, Chardon JW, Li Z, Del Gaudio D, Burmeister M, Gomez CM, et al. Targeted exome analysis identifies the genetic basis of disease in over 50% of patients with a wide range of ataxia-related phenotypes. Genetics in Medicine : Official Journal of the American College of Medical Genetics. PMID 29915382 DOI: 10.1038/S41436-018-0007-7  0.313
2018 McLoughlin HS, Moore LR, Chopra R, Komlo R, McKenzie M, Blumenstein KG, Zhao H, Kordasiewicz HB, Shakkottai VG, Paulson HL. Oligonucleotide therapy mitigates disease in Spinocerebellar Ataxia Type 3 mice. Annals of Neurology. PMID 29908063 DOI: 10.1002/Ana.25264  0.365
2018 Chopra R, Bushart DD, Shakkottai VG. Dendritic potassium channel dysfunction may contribute to dendrite degeneration in spinocerebellar ataxia type 1. Plos One. 13: e0198040. PMID 29847609 DOI: 10.1371/Journal.Pone.0198040  0.442
2018 Bushart DD, Chopra R, Singh V, Murphy GG, Wulff H, Shakkottai VG. Targeting potassium channels to treat cerebellar ataxia. Annals of Clinical and Translational Neurology. 5: 297-314. PMID 29560375 DOI: 10.1002/Acn3.527  0.454
2018 Chopra R, Wasserman AH, Pulst SM, De Zeeuw CI, Shakkottai VG. Protein kinase C activity is a protective modifier of Purkinje neuron degeneration in cerebellar ataxia. Human Molecular Genetics. PMID 29432535 DOI: 10.1093/Hmg/Ddy050  0.366
2018 Bushart DD, Shakkottai VG. Ion channel dysfunction in cerebellar ataxia. Neuroscience Letters. PMID 29421541 DOI: 10.1016/J.Neulet.2018.02.005  0.448
2018 Mundwiler A, Shakkottai VG. Autosomal-dominant cerebellar ataxias. Handbook of Clinical Neurology. 147: 173-185. PMID 29325610 DOI: 10.1016/B978-0-444-63233-3.00012-9  0.374
2017 Kuo PH, Gan SR, Wang J, Lo RY, Figueroa KP, Tomishon D, Pulst SM, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, et al. Dystonia and ataxia progression in spinocerebellar ataxias. Parkinsonism & Related Disorders. PMID 29089256 DOI: 10.1016/J.Parkreldis.2017.10.007  0.303
2017 Dell'Orco JM, Pulst SM, Shakkottai VG. Potassium channel dysfunction underlies Purkinje neuron spiking abnormalities in spinocerebellar ataxia type 2. Human Molecular Genetics. 26: 3935-3945. PMID 29016852 DOI: 10.1093/Hmg/Ddx281  0.464
2017 Paulson HL, Shakkottai VG, Clark HB, Orr HT. Polyglutamine spinocerebellar ataxias - from genes to potential treatments. Nature Reviews. Neuroscience. PMID 28855740 DOI: 10.1038/Nrn.2017.92  0.385
2016 Luo L, Wang J, Lo RY, Figueroa KP, Pulst SM, Kuo PH, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, et al. The Initial Symptom and Motor Progression in Spinocerebellar Ataxias. Cerebellum (London, England). PMID 27848087 DOI: 10.1007/S12311-016-0836-3  0.32
2016 Shakkottai VG, Batla A, Bhatia K, Dauer WT, Dresel C, Niethammer M, Eidelberg D, Raike RS, Smith Y, Jinnah HA, Hess EJ, Meunier S, Hallett M, Fremont R, Khodakhah K, et al. Current Opinions and Areas of Consensus on the Role of the Cerebellum in Dystonia. Cerebellum (London, England). PMID 27734238 DOI: 10.1007/S12311-016-0825-6  0.354
2016 Chung C, Elrick MJ, Dell'Orco JM, Qin ZS, Kalyana-Sundaram S, Chinnaiyan AM, Shakkottai VG, Lieberman AP. Heat Shock Protein Beta-1 Modifies Anterior to Posterior Purkinje Cell Vulnerability in a Mouse Model of Niemann-Pick Type C Disease. Plos Genetics. 12: e1006042. PMID 27152617 DOI: 10.1371/Journal.Pgen.1006042  0.431
2016 Bushart DD, Murphy GG, Shakkottai VG. Precision medicine in spinocerebellar ataxias: treatment based on common mechanisms of disease. Annals of Translational Medicine. 4: 25. PMID 26889478 DOI: 10.3978/j.issn.2305-5839.2016.01.06  0.319
2016 Jones JM, Dionne L, Dell'Orco J, Parent R, Krueger JN, Cheng X, Dib-Hajj SD, Bunton-Stasyshyn RK, Sharkey LM, Dowling JJ, Murphy GG, Shakkottai VG, Shrager P, Meisler MH. Single amino acid deletion in transmembrane segment D4S6 of sodium channel Scn8a (Nav1.6) in a mouse mutant with a chronic movement disorder. Neurobiology of Disease. PMID 26807988 DOI: 10.1016/J.Nbd.2016.01.018  0.457
2015 Dell'Orco JM, Wasserman AH, Chopra R, Ingram MA, Hu YS, Singh V, Wulff H, Opal P, Orr HT, Shakkottai VG. Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 35: 11292-307. PMID 26269637 DOI: 10.1523/Jneurosci.1357-15.2015  0.446
2014 Chopra R, Shakkottai VG. Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia. Future Neurology. 9: 187-196. PMID 25221437 DOI: 10.2217/Fnl.14.6  0.412
2014 Chopra R, Shakkottai VG. The role for alterations in neuronal activity in the pathogenesis of polyglutamine repeat disorders. Neurotherapeutics : the Journal of the American Society For Experimental Neurotherapeutics. 11: 751-63. PMID 24986674 DOI: 10.1007/S13311-014-0289-7  0.407
2014 Shakkottai VG. Physiologic changes associated with cerebellar dystonia. Cerebellum (London, England). 13: 637-44. PMID 24879387 DOI: 10.1007/S12311-014-0572-5  0.325
2014 Luna-Cancalon K, Sikora KM, Pappas SS, Singh V, Wulff H, Paulson HL, Burmeister M, Shakkottai VG. Alterations in cerebellar physiology are associated with a stiff-legged gait in Atcay(ji-hes) mice. Neurobiology of Disease. 67: 140-8. PMID 24727095 DOI: 10.1016/J.Nbd.2014.03.020  0.419
2013 Ashizawa T, Figueroa KP, Perlman SL, Gomez CM, Wilmot GR, Schmahmann JD, Ying SH, Zesiewicz TA, Paulson HL, Shakkottai VG, Bushara KO, Kuo SH, Geschwind MD, Xia G, Mazzoni P, et al. Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study. Orphanet Journal of Rare Diseases. 8: 177. PMID 24225362 DOI: 10.1186/1750-1172-8-177  0.3
2013 Shakkottai VG, Fogel BL. Clinical neurogenetics: autosomal dominant spinocerebellar ataxia. Neurologic Clinics. 31: 987-1007. PMID 24176420 DOI: 10.1016/J.Ncl.2013.04.006  0.374
2013 Costa Mdo C, Luna-Cancalon K, Fischer S, Ashraf NS, Ouyang M, Dharia RM, Martin-Fishman L, Yang Y, Shakkottai VG, Davidson BL, Rodríguez-Lebrón E, Paulson HL. Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease. Molecular Therapy : the Journal of the American Society of Gene Therapy. 21: 1898-908. PMID 23765441 DOI: 10.1038/Mt.2013.144  0.351
2012 Lee YC, Durr A, Majczenko K, Huang YH, Liu YC, Lien CC, Tsai PC, Ichikawa Y, Goto J, Monin ML, Li JZ, Chung MY, Mundwiller E, Shakkottai V, Liu TT, et al. Mutations in KCND3 cause spinocerebellar ataxia type 22. Annals of Neurology. 72: 859-69. PMID 23280837 DOI: 10.1002/Ana.23701  0.363
2011 Shakkottai VG, do Carmo Costa M, Dell'Orco JM, Sankaranarayanan A, Wulff H, Paulson HL. Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 31: 13002-14. PMID 21900579 DOI: 10.1523/Jneurosci.2789-11.2011  0.499
2011 Yu T, Shakkottai VG, Chung C, Lieberman AP. Temporal and cell-specific deletion establishes that neuronal Npc1 deficiency is sufficient to mediate neurodegeneration. Human Molecular Genetics. 20: 4440-51. PMID 21856732 DOI: 10.1093/Hmg/Ddr372  0.413
2010 Elrick MJ, Pacheco CD, Yu T, Dadgar N, Shakkottai VG, Ware C, Paulson HL, Lieberman AP. Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration. Human Molecular Genetics. 19: 837-47. PMID 20007718 DOI: 10.1093/Hmg/Ddp552  0.389
2009 Shakkottai VG, Paulson HL. Physiologic alterations in ataxia: channeling changes into novel therapies. Archives of Neurology. 66: 1196-201. PMID 19822774 DOI: 10.1001/Archneurol.2009.212  0.471
2009 Shakkottai VG, Xiao M, Xu L, Wong M, Nerbonne JM, Ornitz DM, Yamada KA. FGF14 regulates the intrinsic excitability of cerebellar Purkinje neurons. Neurobiology of Disease. 33: 81-8. PMID 18930825 DOI: 10.1016/J.Nbd.2008.09.019  0.38
2007 Wulff H, Kolski-Andreaco A, Sankaranarayanan A, Sabatier JM, Shakkottai V. Modulators of small- and intermediate-conductance calcium-activated potassium channels and their therapeutic indications Current Medicinal Chemistry. 14: 1437-1457. PMID 17584055 DOI: 10.2174/092986707780831186  0.649
2004 Villalobos C, Shakkottai VG, Chandy KG, Michelhaugh SK, Andrade R. SKCa channels mediate the medium but not the slow calcium-activated afterhyperpolarization in cortical neurons. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 24: 3537-42. PMID 15071101 DOI: 10.1523/Jneurosci.0380-04.2004  0.351
2004 Shakkottai VG, Chou CH, Oddo S, Sailer CA, Knaus HG, Gutman GA, Barish ME, LaFerla FM, Chandy KG. Enhanced neuronal excitability in the absence of neurodegeneration induces cerebellar ataxia. The Journal of Clinical Investigation. 113: 582-90. PMID 14966567 DOI: 10.1172/Jci200420216  0.477
2004 Kolski-Andreaco A, Tomita H, Shakkottai VG, Gutman GA, Cahalan MD, Gargus JJ, Chandy KG. SK3-1C, a dominant-negative suppressor of SKCa and IKCa channels. The Journal of Biological Chemistry. 279: 6893-904. PMID 14638680 DOI: 10.1074/Jbc.M311725200  0.619
2003 Tomita H, Shakkottai VG, Gutman GA, Sun G, Bunney WE, Cahalan MD, Chandy KG, Gargus JJ. Novel truncated isoform of SK3 potassium channel is a potent dominant-negative regulator of SK currents: implications in schizophrenia. Molecular Psychiatry. 8: 524-35, 460. PMID 12808432 DOI: 10.1038/Sj.Mp.4001271  0.407
2003 Tomita H, Shakkottai VG, Gutman GA, Sun G, Bunney WE, Cahalan MD, Chandy KG, Gargus JJ. Erratum: Naturally occurring dominant-negative SK3 channel isoform Molecular Psychiatry. 8: 766-766. DOI: 10.1038/Sj.Mp.4001407  0.346
2003 Tomita H, Shakkottai VG, Gutman GA, Sun G, Bunney WE, Cahalan MD, Chandy KG, Gamrgus JJ. Naturally occurring dominant-negative SK3 channel isoform Molecular Psychiatry. 8: 460. DOI: 10.1038/Sj.Mp.4001358  0.376
2001 Shakkottai VG, Regaya I, Wulff H, Fajloun Z, Tomita H, Fathallah M, Cahalan MD, Gargus JJ, Sabatier JM, Chandy KG. Design and characterization of a highly selective peptide inhibitor of the small conductance calcium-activated K+ channel, SkCa2. The Journal of Biological Chemistry. 276: 43145-51. PMID 11527975 DOI: 10.1074/Jbc.M106981200  0.395
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