Vikram Shakkottai, M.D., Ph.D.

Vice Chair for Basic Research, Section Head, Movement Disorders

Peter O'Donnell Jr. Brain Institute

Dedman Family Distinguished Chair in Neurological Disease

Neurology
Cerebellar Ataxia, Inherited and Sporadic Disorders of Cerebellar Dysfunction
Balance Disorders

Biography

Vikram Shakkottai, M.D., Ph.D., obtained his medical degree at the Christian Medical College, Vellore, India, and his Ph.D. at the University of California, Irvine. He received his Neurology Residency training at Washington University/Barnes-Jewish hospital, and was a fellow in movement disorders at the University of Michigan. He joined the full-time faculty at University of Michigan in 2010. He moved to UTSW in 2021.

The long-term goal of Dr. Shakkottai’s clinical and laboratory research is to determine whether alterations in neuronal physiology contribute to motor dysfunction and degeneration in cerebellar ataxia. Work in Dr. Shakkottai’s laboratory has identified dysfunction in the cerebellar circuit common to many murine models of ataxia, with identification of ion channels regulating neuronal intrinsic excitability as key targets for a shared therapeutic strategy.

Dr. Shakkottai's clinical interests include cerebellar ataxia, inherited and sporadic disorders of cerebellar dysfunction, balance disorders, and other movement disorders.

Education & Training

Internship - Washington University St. Louis - Barnes Jewish Hospital (2004-2005), Internal Medicine
Medical School - Christian Medical College, India (1994-2000)
Graduate School - University of California, Irvine (2000-2004), Physiology & Biophysics
Residency - Washington University/Barnes Jewish Hospital (2005-2008)
Fellowship - University of Michigan Medical School (2008-2010), Movement Disorders

Books & Publications

Publications
- Sensitivity of Advanced Magnetic Resonance Imaging to Progression over Six Months in Early Spinocerebellar Ataxia
  Author Collaboration RC, Rezende TJ, Petit E, Park YW, Tezenas du Montcel S, Joers JM, DuBois JM, Moore Arnold H, Povazan M, Banan G, Valabregue R, Ehses P, Faber J, Coupé P, Onyike CU, Barker PB, Schmahmann JD, Ratai EM, Subramony SH, Mareci TH, Bushara KO, Paulson H, Klockgether T, Durr A, Ashizawa T, Lenglet C, Öz G, Rosenthal L, Burns M, Grobe-Einsler M, Oender D, Koyak B, Kimmich O, Roy N, Coarelli G, Ewenczyk C, Heinzmann A, Lallemant P, Hurmic H, Wilmot G, Scorr L, Opal P, Sha S, Santini V, Sampson J, Perlman S, Geschwind M, Nelson A, Dietiker C, Gomez C, Shakkottai V Movement Disorders 2024 Oct 39 1856-1867
- The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale in Spinocerebellar Ataxias
  Selvadurai LP, Perlman SL, Ashizawa T, Wilmot GR, Onyike CU, Rosenthal LS, Shakkottai VG, Paulson HL, Subramony SH, Bushara KO, Kuo SH, Dietiker C, Geschwind MD, Nelson AB, Gomez CM, Opal P, Zesiewicz TA, Hawkins T, Yacoubian TA, Nopoulos PC, Sha SJ, Morrison PE, Figueroa KP, Pulst SM, Schmahmann JD Cerebellum 2024 Aug 23 1411-1425
- SARA captures disparate progression and responsiveness in spinocerebellar ataxias
  Petit E, Schmitz-Hübsch T, Coarelli G, Jacobi H, Heinzmann A, 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, Pulst SM, Subramony SH, Ewenczyk C, Brice A, Durr A, Klockgether T, Ashizawa T, Tezenas du Montcel S Journal of neurology 2024 Jul 271 3743-3753
- ACR Appropriateness Criteria® Dizziness and Ataxia: 2023 Update
  Author Collaboration EP, Wang LL, Thompson TA, Shih RY, Ajam AA, Bulsara K, Burns J, Davis MA, Ivanidze J, Kalnins A, Kuo PH, Ledbetter LN, Pannell JS, Pollock JM, Shakkottai VG, Shih RD, Soares BP, Soderlund KA, Utukuri PS, Woolsey S, Policeni B Journal of the American College of Radiology 2024 Jun 21 S100-S125
- Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias
  Lai RY, Rummey C, Amlang CJ, Lin CY, Chen TX, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Ying SH, Onyike CU, Zesiewicz TA, Bushara KO, Geschwind MD, Figueroa KP, Pulst SM, Subramony SH, Burns MR, Opal P, Duquette A, Ashizawa T, Hamedani AG, Davis MY, Srinivasan SR, Moore LR, Shakkottai VG, Rosenthal LS, Kuo SH Movement Disorders Clinical Practice 2024 May 11 496-503
- Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1
  Tejwani L, Ravindra NG, Lee C, Cheng Y, Nguyen B, Luttik K, Ni L, Zhang S, Morrison LM, Gionco J, Xiang Y, Yoon J, Ro H, Haidery F, Grijalva RM, Bae E, Kim K, Martuscello RT, Orr HT, Zoghbi HY, McLoughlin HS, Ranum LP, Shakkottai VG, Faust PL, Wang S, van Dijk D, Lim J Neuron 2024 Feb 112 362-383.e15
- Deriving early single-rosette brain organoids from human pluripotent stem cells
  Tidball AM, Niu W, Ma Q, Takla TN, Walker JC, Margolis JL, Mojica-Perez SP, Sudyk R, Deng L, Moore SJ, Chopra R, Shakkottai VG, Murphy GG, Yuan Y, Isom LL, Li JZ, Parent JM Stem Cell Reports 2023 Dec 18 2498-2514
- The S-Factor, a New Measure of Disease Severity in Spinocerebellar Ataxia: Findings and Implications
  Selvadurai LP, Perlman SL, Wilmot GR, Subramony SH, Gomez CM, Ashizawa T, Paulson HL, Onyike CU, Rosenthal LS, Sair HI, Kuo SH, Ratai EM, Zesiewicz TA, Bushara KO, Öz G, Dietiker C, Geschwind MD, Nelson AB, Opal P, Yacoubian TA, Nopoulos PC, Shakkottai VG, Figueroa KP, Pulst SM, Morrison PE, Schmahmann JD Cerebellum 2023 Oct 22 790-809
- Targeting Ion Channels and Purkinje Neuron Intrinsic Membrane Excitability as a Therapeutic Strategy for Cerebellar Ataxia
  Huang H, Shakkottai VG Life 2023 Jun 13
- Genome-wide structural variant analysis identifies risk loci for non-Alzheimer's dementias
  Author Collaboration PC, Author Collaboration TA, Author Collaboration IL, Author Collaboration IA, Kaivola K, Chia R, Ding J, Rasheed M, Fujita M, Menon V, Walton RL, Collins RL, Billingsley K, Brand H, Talkowski M, Zhao X, Dewan R, Stark A, Ray A, Solaiman S, Alvarez Jerez P, Malik L, Dawson TM, Rosenthal LS, Albert MS, Pletnikova O, Troncoso JC, Masellis M, Keith J, Black SE, Ferrucci L, Resnick SM, Tanaka T, Soltis AR, Viollet C, Sukumar G, Alba C, Lott N, McGrath Martinez E, Tuck M, Singh J, Bacikova D, Zhang X, Hupalo DN, Adeleye A, Wilkerson MD, Pollard HB, Dalgard CL, Gan-Or Z, Rogaeva E, Brice A, Lesage S, Xiromerisiou G, Shakkottai VG Cell Genomics 2023 Jun 3
- Neuronal activity regulates Matrin 3 abundance and function in a calcium-dependent manner through calpain-mediated cleavage and calmodulin binding
  Malik AM, Wu JJ, Gillies CA, Doctrove QA, Li X, Huang H, Tank EH, Shakkottai VG, Barmada S Proceedings of the National Academy of Sciences of the United States of America 2023 Apr 120
- SCAview: an Intuitive Visual Approach to the Integrative Analysis of Clinical Data in Spinocerebellar Ataxias
  Author Collaboration Es, Author Collaboration Es, Author Collaboration Rs, Author Collaboration Cs, Author Collaboration SR, Uebachs M, Wegner P, Schaaf S, Kugai S, Jacobi H, Kuo SH, Ashizawa T, Fluck J, du Montcel ST, Bauer P, Giunti P, Cook A, Labrum R, Parkinson MH, Durr A, Brice A, Charles P, Marelli C, Mariotti C, Nanetti L, Panzeri M, Rakowicz M, Sulek A, Sobanska A, Schmitz-Hübsch T, Schöls L, Hengel H, Baliko L, Melegh B, Filla A, Antenora A, Infante J, Berciano J, van de Warrenburg BP, Timmann D, Szymanski S, Boesch S, Kang JS, Pandolfo M, Schulz JB, Molho S, Diallo A, Grobe-Einsler M, Önder D, Raposo M, Vasconcelos J, Lima M, de Almeida LP, Silva P, Shakkottai VG Cerebellum 2023
- Discovery of Novel Activators of Large-Conductance Calcium-Activated Potassium Channels for the Treatment of Cerebellar Ataxia^S
  Srinivasan SR, Huang H, Chang WC, Nasburg J, Nguyen H, Strassmaier T, Wulff H, Shakkottai VG Molecular Pharmacology 2022 Jul 102 17-28
- Vulnerability of Human Cerebellar Neurons to Degeneration in Ataxia-Causing Channelopathies
  Bushart DD, Shakkottai VG Frontiers in Systems Neuroscience 2022 Jun 16
- Genetic evaluation of dementia with Lewy bodies implicates distinct disease subgroups
  Kaivola K, Shah Z, Chia R, Black SE, Gan-Or Z, Keith J, Masellis M, Rogaeva E, Brice A, Lesage S, Xiromerisiou G, Calvo A, Canosa A, Chio A, Logroscino G, Mora G, Krüger R, May P, Alcolea D, Clarimon J, Fortea J, Gonzalez-Aramburu I, Infante J, Lage C, Lleó A, Pastor P, Sanchez-Juan P, Brett F, Aarsland D, Al-Sarraj S, Attems J, Gentleman S, Hardy JA, Hodges AK, Love S, Mckeith IG, Morris CM, Morris HR, Palmer L, Pickering-Brown S, Ryten M, Thomas AJ, Troakes C, Albert MS, Barrett MJ, Beach TG, Bekris LM, Bennett DA, Boeve BF, Dalgard CL, Dawson TM, Dickson DW, Faber K, Ferman T, Ferrucci L, Flanagan ME, Foroud TM, Ghetti B, Gibbs JR, Goate A, Goldstein DS, Graff-Radford NR, Kaufmann H, Kukull WA, Leverenz JB, Mao Q, Masliah E, Monuki E, Newell KL, Palma JA, Pletnikova O, Renton AE, Resnick SM, Rosenthal LS, Ross OA, Scherzer CR, Serrano GE, Shakkottai VG, Sidransky E, Tanaka T, Topol E, Torkamani A, Troncoso JC, Woltjer R, Wszolek ZK, Scholz SW Brain 2022 May 145 1757-1762
- Preliminary Study of Vibrotactile Feedback during Home-Based Balance and Coordination Training in Individuals with Cerebellar Ataxia
  Jabri S, Bushart DD, Kinnaird C, Bao T, Bu A, Shakkottai VG, Sienko KH Sensors 2022 May 22
- THAP1 modulates oligodendrocyte maturation by regulating ECM degradation in lysosomes
  Yellajoshyula D, Pappas SS, Rogers AE, Choudhury B, Reed X, Ding J, Cookson MR, Shakkottai VG, Giger RJ, Dauer WT Proceedings of the National Academy of Sciences of the United States of America 2021 Aug 118
- Genome sequencing analysis identifies new loci associated with Lewy body dementia and provides insights into its genetic architecture
  Author Collaboration TA, Chia R, Sabir MS, Bandres-Ciga S, Saez-Atienzar S, Reynolds RH, Gustavsson E, Walton RL, Ahmed S, Viollet C, Ding J, Makarious MB, Diez-Fairen M, Portley MK, Shah Z, Abramzon Y, Hernandez DG, Blauwendraat C, Stone DJ, Eicher J, Parkkinen L, Ansorge O, Clark L, Honig LS, Marder K, Lemstra A, St George-Hyslop P, Londos E, Morgan K, Lashley T, Warner TT, Jaunmuktane Z, Galasko D, Santana I, Tienari PJ, Myllykangas L, Oinas M, Cairns NJ, Morris JC, Halliday GM, Van Deerlin VM, Trojanowski JQ, Grassano M, Calvo A, Mora G, Canosa A, Floris G, Bohannan RC, Brett F, Gan-Or Z, Shakkottai VG Nature genetics 2021 Mar 53 294-303
- A Chlorzoxazone-Baclofen Combination Improves Cerebellar Impairment in Spinocerebellar Ataxia Type 1
  Bushart DD, Huang H, Man LJ, Morrison LM, Shakkottai VG Movement Disorders 2021 Mar 36 622-631
- Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis
  Author Collaboration TP, Author Collaboration TA, Author Collaboration TF, Author Collaboration TG, Author Collaboration TI, Author Collaboration TI, Author Collaboration TI, Author Collaboration tU, Dewan R, Chia R, Ding J, Hickman RA, Stein TD, Abramzon Y, Ahmed S, Sabir MS, Portley MK, Tucci A, Ibáñez K, Shankaracharya FN, Keagle P, Rossi G, Caroppo P, Tagliavini F, Waldo ML, Johansson PM, Nilsson CF, Adeleye A, Alba C, Bacikova D, Hupalo DN, Martinez EM, Pollard HB, Sukumar G, Soltis AR, Tuck M, Zhang X, Wilkerson MD, Smith BN, Ticozzi N, Fallini C, Gkazi AS, Topp SD, Kost J, Scotter EL, Kenna KP, Miller JW, Tiloca C, Vance C, Danielson EW, Troakes C, Colombrita C, Al-Sarraj S, Lewis EA, King A, Calini D, Pensato V, Shakkottai V Neuron 2021 Feb 109 448-460.e4
- Antisense Oligonucleotide Therapy Targeted Against ATXN3 Improves Potassium Channel–Mediated Purkinje Neuron Dysfunction in Spinocerebellar Ataxia Type 3
  Bushart DD, Zalon AJ, Zhang H, Morrison LM, Guan Y, Paulson HL, Shakkottai VG, McLoughlin HS Cerebellum 2021 Feb 20 41-53
- Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1
  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 Human molecular genetics 2020 Oct 29 3249-3265
- Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3
  Prudencio M, Garcia-Moreno H, Jansen-West KR, AL-Shaikh RH, Gendron TF, Heckman MG, Spiegel MR, Carlomagno Y, Daughrity LM, Song Y, Dunmore JA, Byron N, Oskarsson B, Nicholson KA, Staff NP, Gorcenco S, Puschmann A, Lemos J, Januário C, LeDoux MS, Friedman JH, Polke J, Labrum R, Shakkottai V, McLoughlin HS, Paulson HL, Konno T, Onodera O, Ikeuchi T, Tada M, Kakita A, Fryer JD, Karremo C, Gomes I, Caviness JN, Pittelkow MR, Aasly J, Pfeiffer RF, Veerappan V, Eggenberger ER, Freeman WD, Huang JF, Uitti RJ, Wierenga KJ, Marin Collazo IV, Tipton PW, van Gerpen JA, van Blitterswijk M, Bu G, Wszolek ZK, Giunti P, Petrucelli L Science translational medicine 2020 Oct 12
- In Vivo Molecular Signatures of Cerebellar Pathology in Spinocerebellar Ataxia Type 3
  Costa Md, Radzwion M, McLoughlin HS, Ashraf NS, Fischer S, Shakkottai VG, Maciel P, Paulson HL, Öz G Movement Disorders 2020 Oct 35 1774-1786
- Dysphagia in spinocerebellar ataxias type 1, 2, 3 and 6
  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, Bushara K, Geschwind M, Xia G, Subramony SH, Ashizawa T, Troche MS, Kuo SH Journal of the Neurological Sciences 2020 Aug 415
- Multiple system atrophy pathology is associated with primary Sjögren’s syndrome
  Conway KS, Camelo-Piragua S, Fisher-Hubbard A, Perry WR, Shakkottai VG, Venneti S JCI Insight 2020 Aug 5
- The impact of ethnicity on the clinical presentations of spinocerebellar ataxia type 3
  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, Rosenthal L, Ashizawa T, Pulst SM, Wang N, Kuo SH Parkinsonism and Related Disorders 2020 Mar 72 37-43
- Alanyl-tRNA Synthetase 2 (AARS2)-Related Ataxia Without Leukoencephalopathy
  Kuo ME, Antonellis A, Shakkottai VG Cerebellum 2020 Feb 19 154-160
- Nicotinamide Pathway-Dependent Sirt1 Activation Restores Calcium Homeostasis to Achieve Neuroprotection in Spinocerebellar Ataxia Type 7
  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, La Spada AR Neuron 2020 Feb 105 630-644.e9
- The inherited ataxias
  Rosenberg RN, Khemani P, Shakkottai VG 2020 Jan 75-97
- Synthetic high-density lipoprotein nanoparticles for the treatment of Niemann-Pick diseases
  Schultz ML, Fawaz MV, Azaria RD, Hollon TC, Liu EA, Kunkel TJ, Halseth TA, Krus KL, Ming R, Morin EE, McLoughlin HS, Bushart DD, Paulson HL, Shakkottai VG, Orringer DA, Schwendeman AS, Lieberman AP BMC medicine 2019 Nov 17
- Moving Towards Therapy in SCA1: Insights from Molecular Mechanisms, Identification of Novel Targets, and Planning for Human Trials
  Srinivasan SR, Shakkottai VG Neurotherapeutics 2019 Oct 16 999-1008
- Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line
  Moore LR, Keller L, Bushart DD, Delatorre RG, Li D, McLoughlin HS, do Carmo Costa M, Shakkottai VG, Smith GD, Paulson HL Stem Cell Research 2019 Aug 39
- Tremor in the Degenerative Cerebellum: Towards the Understanding of Brain Circuitry for Tremor
  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, Subramony SH, Ashizawa T, Kuo SH Cerebellum 2019 Jun 18 519-526
- COQ4 Mutation Leads to Childhood-Onset Ataxia Improved by CoQ10 Administration
  Caglayan AO, Gumus H, Sandford E, Kubisiak TL, Ma Q, Ozel AB, Per H, Li JZ, Shakkottai VG, Burmeister M Cerebellum 2019 Jun 18 665-669
- Expanding the genetic basis of ataxia
  Shakkottai V, Paulson H Nature genetics 2019 Apr 51 580-581
- Generation of Spinocerebellar Ataxia Type 2 induced pluripotent stem cell lines, CHOPi002-A and CHOPi003-A, from patients with abnormal CAG repeats in the coding region of the ATXN2 gene
  Maguire JA, Gagne AL, Gonzalez-Alegre P, Davidson BL, Shakkottai V, Gadue P, French DL Stem Cell Research 2019 Jan 34
- Polyglutamine Repeats in Neurodegenerative Diseases
  Lieberman AP, Shakkottai VG, Albin RL Annual Review of Pathology: Mechanisms of Disease 2019 14 1-27
- Ion channel dysfunction in cerebellar ataxia
  Bushart DD, Shakkottai VG Neuroscience letters 2019 Jan 688 41-48
- Targeted exome analysis identifies the genetic basis of disease in over 50% of patients with a wide range of ataxia-related phenotypes
  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, Waggoner DJ, Das S Genetics in Medicine 2019 Jan 21 195-206
- MTSS1/Src family kinase dysregulation underlies multiple inherited ataxias
  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, Oro AE Proceedings of the National Academy of Sciences of the United States of America 2018 Dec 115 E12407-E12416
- Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
  Schultz ML, Krus KL, Kaushik S, Dang D, Chopra R, Qi L, Shakkottai VG, Cuervo AM, Lieberman AP Nature communications 2018 Dec 9
- Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice
  McLoughlin HS, Moore LR, Chopra R, Komlo R, McKenzie M, Blumenstein KG, Zhao H, Kordasiewicz HB, Shakkottai VG, Paulson HL Annals of Neurology 2018 Jul 84 64-77
- Mutations in VPS13D lead to a new recessive ataxia with spasticity and mitochondrial defects
  Seong E, Insolera R, Dulovic M, Kamsteeg EJ, Trinh J, Brüggemann N, Sandford E, Li S, Ozel AB, Li JZ, Jewett T, Kievit AJ, Münchau A, Shakkottai V, Klein C, Collins CA, Lohmann K, van de Warrenburg BP, Burmeister M Annals of Neurology 2018 Jun 83 1075-1088
- Dendritic potassium channel dysfunction may contribute to dendrite degeneration in spinocerebellar ataxia type 1
  Chopra R, Bushart DD, Shakkottai VG PloS one 2018 May 13
- Protein kinase C activity is a protective modifier of Purkinje neuron degeneration in cerebellar ataxia
  Chopra R, Wasserman AH, Pulst SM, De Zeeuw CI, Shakkottai VG Human molecular genetics 2018 Apr 27 1396-1410
- Targeting potassium channels to treat cerebellar ataxia
  Bushart DD, Chopra R, Singh V, Murphy GG, Wulff H, Shakkottai VG Annals of Clinical and Translational Neurology 2018 Mar 5 297-314
- C9orf72 repeat expansions as genetic modifiers for depression in spinocerebellar ataxias
  Figueroa KP, Gan SR, Perlman S, Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind M, Xia G, Subramony SH, Ashizawa T, Pulst SM, Kuo SH Movement Disorders 2018 Mar 33 497-498
- Autosomal-dominant cerebellar ataxias
  Mundwiler A, Shakkottai VG 2018 173-185
- Dystonia and ataxia progression in spinocerebellar ataxias
  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, Bushara K, Geschwind MD, Xia G, Subramony SH, Ashizawa T, Kuo SH Parkinsonism and Related Disorders 2017 Dec 45 75-80
- Potassium channel dysfunction underlies Purkinje neuron spiking abnormalities in spinocerebellar ataxia type 2
  Dell'Orco JM, Pulst SM, Shakkottai VG Human molecular genetics 2017 Oct 26 3935-3945
- Postural tremor and ataxia progression in spinocerebellar ataxias
  Gan SR, Wang J, Figueroa KP, Pulst SM, Tomishon D, Lee D, Perlman S, Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind MD, Xia G, Subramony SH, Ashizawa T, Kuo SH Tremor and Other Hyperkinetic Movements 2017 Oct 7
- Polyglutamine spinocerebellar ataxias-from genes to potential treatments
  Paulson HL, Shakkottai VG, Clark HB, Orr HT Nature Reviews Neuroscience 2017 Sep 18 613-626
- The Initial Symptom and Motor Progression in Spinocerebellar Ataxias
  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, Geschwind M, Xia G, Subramony SH, Ashizawa T, Kuo SH Cerebellum 2017 Jun 16 615-622
- Current Opinions and Areas of Consensus on the Role of the Cerebellum in Dystonia
  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, LeDoux MS, Popa T, Gallea C, Lehericy S, Bostan AC, Strick PL Cerebellum 2017 Apr 16 577-594
- Single amino acid deletion in transmembrane segment D4S6 of sodium channel Scn8a (Na_v1.6) in a mouse mutant with a chronic movement disorder
  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 Neurobiology of Disease 2016 May 89 36-45
- Heat Shock Protein Beta-1 Modifies Anterior to Posterior Purkinje Cell Vulnerability in a Mouse Model of Niemann-Pick Type C Disease
  Chung C, Elrick MJ, Dell’Orco JM, Qin ZS, Kalyana-Sundaram S, Chinnaiyan AM, Shakkottai VG, Lieberman AP PLoS genetics 2016 May 12
- Depression and clinical progression in spinocerebellar ataxias
  Lo RY, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez C, Schmahmann J, Paulson H, Shakkottai VG, Ying S, Zesiewicz T, Bushara K, Geschwind M, Xia G, Yu JT, Lee LE, Ashizawa T, Subramony SH, Kuo SH Parkinsonism and Related Disorders 2016 Jan 22 87-92
- Precision medicine in spinocerebellar ataxias: Treatment based on common mechanisms of disease
  Bushart DD, Murphy GG, Shakkottai VG Annals of Translational Medicine 2016 Jan 4
- Neuronal atrophy early in degenerative ataxia is a compensatory mechanism to regulate membrane excitability
  Dell’Orco JM, Wasserman AH, Chopra R, Ingram MA, Hu YS, Singh V, Wulff H, Opal P, Orr HT, Shakkottai VG Journal of Neuroscience 2015 Aug 35 11292-11307
- Clinical evaluation of eye movements in spinocerebellar ataxias: A prospective multicenter study
  Moscovich M, Okun MS, Favilla C, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez C, Schmahmann J, Paulson H, Shakkottai V, Ying S, Zesiewicz T, Kuo SH, Mazzoni P, Bushara K, Xia G, Ashizawa T, Subramony SH Journal of Neuro-Ophthalmology 2015 Mar 35 16-21
- Coenzyme Q10 and spinocerebellar ataxias
  Lo RY, Figueroa KP, Pulst SM, Lin CY, Perlman S, Wilmot G, Gomez C, Schmahmann J, Paulson H, Shakkottai VG, Ying S, Zesiewicz T, Bushara K, Geschwind M, Xia G, Subramony SH, Ashizawa T, Kuo SH Movement Disorders 2015 Feb 30 214-220
- The Role for Alterations in Neuronal Activity in the Pathogenesis of Polyglutamine Repeat Disorders
  Chopra R, Shakkottai VG Neurotherapeutics 2014 Oct 11 751-763
- Physiologic Changes Associated with Cerebellar Dystonia
  Shakkottai VG Cerebellum 2014 Oct 13 637-644
- Alterations in cerebellar physiology are associated with a stiff-legged gait in Atcay^ji-hes mice
  Luna-Cancalon K, Sikora KM, Pappas SS, Singh V, Wulff H, Paulson HL, Burmeister M, Shakkottai VG Neurobiology of Disease 2014 Jul 67 140-148
- Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia
  Chopra R, Shakkottai VG Future Neurology 2014 Mar 9 187-196
- Clinical Neurogenetics: Autosomal Dominant Spinocerebellar Ataxia
  Shakkottai VG, Fogel BL Neurologic Clinics 2013 Nov 31 987-1007
- Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease
  Do Carmo Costa M, 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 Molecular Therapy 2013 Oct 21 1898-1908
- Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; A prospective observational study
  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, Krischer JP, Cuthbertson D, Holbert AR, Ferguson JH, Pulst SM, Subramony S Orphanet Journal of Rare Diseases 2013 8
- Mutations in KCND3 cause spinocerebellar ataxia type 22
  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, Tesson C, Lu YC, Brice A, Tsuji S, Burmeister M, Stevanin G, Soong BW Annals of Neurology 2012 Dec 72 859-869
- Temporal and cell-specific deletion establishes that neuronal Npc1 deficiency is sufficient to mediate neurodegeneration
  Yu T, Shakkottai VG, Chung C, Lieberman AP Human molecular genetics 2011 Nov 20 4440-4451
- Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3
  Shakkottai VG, Costa Md, Dell'Orco JM, Sankaranarayanan A, Wulff H, Paulson HL Journal of Neuroscience 2011 Sep 31 13002-13014
- Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration
  Elrick MJ, Pacheco CD, Yu T, Dadgar N, Shakkottai VG, Ware C, Paulson HL, Lieberman AP Human molecular genetics 2010 Mar 19 837-847
- Physiologic alterations in ataxia: Channeling changes into novel therapies
  Shakkottai VG, Paulson HL Archives of neurology 2009 66 1196-1201
- FGF14 regulates the intrinsic excitability of cerebellar Purkinje neurons
  Shakkottai VG, Xiao M, Xu L, Wong M, Nerbonne JM, Ornitz DM, Yamada KA Neurobiology of Disease 2009 Jan 33 81-88
- Modulators of small- and intermediate-conductance calcium-activated potassium channels and their therapeutic indications
  Wulff H, Kolski-Andreaco A, Sankaranarayanan A, Sabatier JM, Shakkottai V Current Medicinal Chemistry 2007 Jun 14 1437-1457
- SK_Ca Channels Mediate the Medium but not the Slow Calcium-Activated Afterhyperpolarization in Cortical Neurons
  Villalobos C, Shakkottai VG, Chandy KG, Michelhaugh SK, Andrade R Journal of Neuroscience 2004 Apr 24 3537-3542
- SK3-1C, a Dominant-negative Suppressor of SK_Ca and IK _Ca Channels
  Kolski-Andreaco A, Tomita H, Shakkottai VG, Gutman GA, Cahalan MD, Gargus JJ, Chandy KG Journal of Biological Chemistry 2004 Feb 279 6893-6904
- Enhanced neuronal excitability in the absence of neurodegeneration induces cerebellar ataxia
  Shakkottai VG, Chou CH, Oddo S, Sailer CA, Knaus HG, Gutman GA, Barish ME, LaFerla FM, Chandy KG Journal of Clinical Investigation 2004 Feb 113 582-590
- Novel truncated isoform of SK3 potassium channel is a potent dominant-negative regulator of SK currents: Implications in schizophrenia
  Tomita H, Shakkottai VG, Gutman GA, Sun G, Bunney WE, Cahalan MD, Chandy KG, Gargus JJ Molecular psychiatry 2003 8 524-535
- Erratum: Naturally occurring dominant-negative SK3 channel isoform (Molecular Psychiatry (2003) 8 (766))
  Tomita H, Shakkottai VG, Gutman GA, Sun G, Bunney WE, Cahalan MD, Chandy KG, Gargus JJ Molecular psychiatry 2003 8 766
- Naturally occurring dominant-negative SK3 channel isoform
  Tomita H, Shakkottai VG, Gutman GA, Sun G, Bunney WE, Cahalan MD, Chandy KG, Gamrgus JJ Molecular psychiatry 2003 8 460
- Gramicidin S: A peptide model for protein glycation and reversal of glycation using nucleophilic amines
  Shakkottai VG, Sudha R, Balaram P Journal of Peptide Research 2002 60 112-120
- Design and Characterization of a Highly Selective Peptide Inhibitor of the Small Conductance Calcium-activated K⁺ Channel, SkCa2
  Shakkottai VG, Regaya I, Wulff H, Fajloun Z, Tomita H, Fathallah M, Cahalan MD, Gargus JJ, Sabatier JM, Chandy KG Journal of Biological Chemistry 2001 Nov 276 43145-43151
- Genomic organization and promoter analysis of human KCNN3 gene
  Sun G, Tomita H, Shakkottai VG, Gargus JJ Journal of Human Genetics 2001 46 463-470

Clinical Focus

Cerebellar Ataxia, Inherited and Sporadic Disorders of Cerebellar Dysfunction
Balance Disorders
Movement Disorders

Vikram Shakkottai, M.D., Ph.D.

Biography

Education & Training

Books & Publications

Clinical Focus

Neurology Clinic - Movement Disorders