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Holly B. Kordasiewicz · SciRah

Researcher profile · Semantic Scholar

Holly B. Kordasiewicz

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Holly B. Kordasiewicz — researcher profile on SciRah

h-index: 37
Works: 89
Citations: 7,849

Most-cited papers

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
M. Polymenidou, C. Lagier-Tourenne, Kasey R. Hutt, Stephanie C. Huelga +13 more
2011Nature Neuroscience1,322 citations Open access
Semantic Scholar
Sustained therapeutic reversal of Huntington’s disease by transient repression of huntingtin synthesis
Holly B. Kordasiewicz, L. Stanek, E. Wancewicz, C. Mazur +9 more
2012Neuron705 citations Open access
Semantic Scholar
Targeting Huntingtin Expression in Patients with Huntington's Disease.
S. Tabrizi, B. Leavitt, G. Landwehrmeyer, E. Wild +18 more
2019New England Journal of Medicine548 citations Open access
Semantic Scholar
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43
Eveline S. Arnold, Shuo-Chien Ling, Stephanie C. Huelga, C. Lagier-Tourenne +14 more
2013Proceedings of the National Academy of Sciences of the United States of America471 citations Open access
Semantic Scholar
Tau Reduction Prevents Neuronal Loss and Reverses Pathological Tau Deposition and Seeding in Mice with Tauopathy
Sarah L. DeVos, Rebecca L. Miller, Kathleen M. Schoch, Brandon B. Holmes +12 more
2017Science Translational Medicine460 citations Open access
Semantic Scholar
Tau-targeting antisense oligonucleotide MAPTRx in mild Alzheimer’s disease: a phase 1b, randomized, placebo-controlled trial
C. Mummery, A. Börjesson-Hanson, D. Blackburn, E. Vijverberg +23 more
2023Nature Medicine284 citations Open access
Semantic Scholar
Single-Stranded RNAs Use RNAi to Potently and Allele-Selectively Inhibit Mutant Huntingtin Expression
Dongbo Yu, H. Pendergraff, Jing Liu, Holly B. Kordasiewicz +6 more
2012Cell269 citations Open access
Semantic Scholar
Antisense oligonucleotides extend survival and reverse decrement in muscle response in ALS models
Alexander McCampbell, Tracy A. Cole, A. Wegener, G. Tomassy +19 more
2018Journal of Clinical Investigation235 citations Open access
Semantic Scholar
Caveolin Proteins Are Essential for Distinct Effects of Membrane Estrogen Receptors in Neurons
Marissa I. Boulware, Holly B. Kordasiewicz, P. Mermelstein
2007Journal of Neuroscience200 citations Open access
Semantic Scholar
LRRK2 Antisense Oligonucleotides Ameliorate α-Synuclein Inclusion Formation in a Parkinson’s Disease Mouse Model
Hien T. Zhao, N. John, V. Delic, Karli Ikeda-Lee +6 more
2017Molecular Therapy: Nucleic Acids192 citations Open access
Semantic Scholar
Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS
Michael E. Østergaard, Amber L. Southwell, Holly B. Kordasiewicz, A. Watt +8 more
2013Nucleic Acids Research155 citations Open access
Semantic Scholar
C-termini of P/Q-type Ca2+ channel alpha1A subunits translocate to nuclei and promote polyglutamine-mediated toxicity.
Holly B. Kordasiewicz, Randall M. Thompson, H. Clark, Christopher M. Gomez
2006Human Molecular Genetics155 citations Open access
Semantic Scholar
Antisense oligonucleotides extend survival of prion-infected mice
G. J. Raymond, Hien T. Zhao, Brent Race, L. Raymond +18 more
2019JCI Insight146 citations Open access
Semantic Scholar
Oligonucleotide therapy mitigates disease in Spinocerebellar Ataxia Type 3 mice
Hayley S. McLoughlin, Lauren R. Moore, Ravi Chopra, Robert Komlo +6 more
2018Annals of Neurology142 citations Open access
Semantic Scholar
PMP22 antisense oligonucleotides reverse Charcot-Marie-Tooth disease type 1A features in rodent models
Hien T. Zhao, S. Damle, Karli Ikeda-Lee, Steven G Kuntz +9 more
2017Journal of Clinical Investigation141 citations Open access
Semantic Scholar
Tcf4 Regulates Synaptic Plasticity, DNA Methylation, and Memory Function
Andrew J. Kennedy, E. Rahn, Brynna S. Paulukaitis, Katherine E. Savell +14 more
2016Cell Reports138 citations Open access
Semantic Scholar
In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides.
Amber L. Southwell, N. Skotte, Holly B. Kordasiewicz, Michael E. Østergaard +12 more
2014Molecular Therapy137 citations Open access
Semantic Scholar
Prion protein lowering is a disease-modifying therapy across prion disease stages, strains and endpoints
E. Minikel, Hien T. Zhao, Jason Lê, Jill O'Moore +20 more
2020bioRxiv131 citations Open access
Semantic Scholar
α-Synuclein antisense oligonucleotides as a disease-modifying therapy for Parkinson’s disease
Tracy A. Cole, Hien T. Zhao, T. Collier, I. Sandoval +21 more
2021JCI Insight126 citations Open access
Semantic Scholar
Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models
Lauren R. Moore, Gautam Rajpal, Ian T. Dillingham, Maya Qutob +6 more
2017Molecular Therapy: Nucleic Acids124 citations Open access
Semantic Scholar
Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles.
Jillian Friedrich, Holly B. Kordasiewicz, Brennon L. O'Callaghan, Hillary P. Handler +16 more
2018JCI Insight121 citations Open access
Semantic Scholar
The atlas of RNase H antisense oligonucleotide distribution and activity in the CNS of rodents and non-human primates following central administration
P. Jafar-nejad, Berit Powers, Armand Soriano, Hien T. Zhao +10 more
2020bioRxiv113 citations Open access
Semantic Scholar
Allele-Specific Suppression of Mutant Huntingtin Using Antisense Oligonucleotides: Providing a Therapeutic Option for All Huntington Disease Patients
N. Skotte, Amber L. Southwell, Michael E. Østergaard, J. Carroll +12 more
2014PLoS ONE113 citations Open access
Semantic Scholar
Huntingtin suppression restores cognitive function in a mouse model of Huntington’s disease
Amber L. Southwell, Holly B. Kordasiewicz, D. Langbehn, N. H. Skotte +15 more
2018Science Translational Medicine112 citations Open access
Semantic Scholar
Gene suppression strategies for dominantly inherited neurodegenerative diseases: lessons from Huntington's disease and spinocerebellar ataxia.
Megan S. Keiser, Holly B. Kordasiewicz, J. McBride
2016Human Molecular Genetics105 citations Open access
Semantic Scholar
Brain pharmacology of intrathecal antisense oligonucleotides revealed through multimodal imaging.
C. Mazur, Berit Powers, K. Zasadny, Jenna M. Sullivan +20 more
2019JCI Insight103 citations Open access
Semantic Scholar
Antisense Drugs Make Sense for Neurological Diseases
C. F. Bennett, Holly B. Kordasiewicz, Don W. Cleveland
2020Annual Review of Pharmacology and Toxicology95 citations Open access
Semantic Scholar
Intrathecal administration of antisense oligonucleotide against p38α but not p38β MAP kinase isoform reduces neuropathic and postoperative pain and TLR4-induced pain in male mice
Xin Luo, B. Fitzsimmons, Apoorva Mohan, Lin-Lin Zhang +3 more
2017Brain, behavior, and immunity83 citations Open access
Semantic Scholar
MicroRNA Profiling Reveals Marker of Motor Neuron Disease in ALS Models
Mariah L. Hoye, Erica D. Koval, A. Wegener, T. Hyman +13 more
2017Journal of Neuroscience76 citations Open access
Semantic Scholar
Huntingtin-Lowering Therapies for Huntington Disease: A Review of the Evidence of Potential Benefits and Risks.
B. Leavitt, Holly B. Kordasiewicz, S. Schobel
2020JAMA Neurology62 citations
Semantic Scholar
Molecular pathogenesis of spinocerebellar ataxia type 6
Holly B. Kordasiewicz, Christopher M. Gomez
2007Neurotherapeutics54 citations Open access
Semantic Scholar
Gys1 antisense therapy rescues neuropathological bases of murine Lafora disease
S. Ahonen, S. Nitschke, Tamar R. Grossman, Holly B. Kordasiewicz +6 more
2021bioRxiv47 citations Open access
Semantic Scholar
Characterization of the Prion Protein Binding Properties of Antisense Oligonucleotides
A. Reidenbach, E. Minikel, Hien T. Zhao, Stacy Guzman +5 more
2019bioRxiv45 citations Open access
Semantic Scholar
Antisense oligonucleotide-mediated MSH3 suppression reduces somatic CAG repeat expansion in Huntington's disease iPSC-derived striatal neurons.
Emma L Bunting, Jasmine Donaldson, S. Cumming, Jessica Olive +17 more
2025Science Translational Medicine40 citations Open access
Semantic Scholar
Alternative processing of human HTT mRNA with implications for Huntington’s disease therapeutics
Sandra Fienko, C. Landles, Kirupa Sathasivam, Sean J McAteer +10 more
2022Brain : a journal of neurology38 citations Open access
Semantic Scholar
Antisense oligonucleotides selectively suppress target RNA in nociceptive neurons of the pain system and can ameliorate mechanical pain
Apoorva Mohan, B. Fitzsimmons, Hien T. Zhao, Yuhong Jiang +3 more
2018Pain38 citations
Semantic Scholar
Translating Antisense Technology into a Treatment for Huntington's Disease.
R. Lane, Anne V. Smith, Tiffany L. Baumann, M. Gleichmann +3 more
2018Methods in molecular biology38 citations
Semantic Scholar
A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles
Amber L. Southwell, N. Skotte, Erika B. Villanueva, Michael E. Østergaard +18 more
2017Human Molecular Genetics37 citations Open access
Semantic Scholar
A single-cell map of antisense oligonucleotide activity in the brain
M. Mortberg, Juliana E. Gentile, Naeem M. Nadaf, Charles R. Vanderburg +10 more
2023Nucleic Acids Research34 citations Open access
Semantic Scholar
Convective forces increase rostral delivery of intrathecal radiotracers and antisense oligonucleotides in the cynomolgus monkey nervous system
Jenna M. Sullivan, C. Mazur, Daniel A. Wolf, Laura L. Horky +12 more
2020Journal of Translational Medicine34 citations Open access
Semantic Scholar
Regional variability and genotypic and pharmacodynamic effects on PrP concentration in the CNS
M. Mortberg, Hien T. Zhao, A. Reidenbach, Juliana E. Gentile +16 more
2022JCI Insight29 citations Open access
Semantic Scholar
Effects of IONIS-HTTRx in Patients with Early Huntington’s Disease, Results of the First HTT-Lowering Drug Trial (CT.002)
S. Tabrizi, B. Leavitt, Holly B. Kordasiewicz, C. Czech +8 more
2018Neurology29 citations
Semantic Scholar
Alpha-synuclein antisense oligonucleotides as a disease-modifying therapy for Parkinson’s disease
Tracy A. Cole, Hien T. Zhao, Timothy J. Collier, I. Sandoval +21 more
2019bioRxiv27 citations Open access
Semantic Scholar
Gene-targeted therapies for the central nervous system.
T. Miller, Richard A. G. Smith, Holly B. Kordasiewicz, B. Kaspar
2008Archives of Neurology27 citations Open access
Semantic Scholar
Dominant-negative suppression of Cav2.1 currents by alpha(1)2.1 truncations requires the conserved interaction domain for beta subunits.
Robert S. Raike, Holly B. Kordasiewicz, Randall M. Thompson, Christopher M. Gomez
2007Molecular and cellular neurosciences26 citations Open access
Semantic Scholar
Design of the First-in-Human Study of IONIS-MAPTRx, a Tau-lowering Antisense Oligonucleotide, in Patients With Alzheimer Disease (S2.006)
L. Mignon, Holly B. Kordasiewicz, R. Lane, Anne V. Smith +6 more
2018Neurology24 citations
Semantic Scholar
Antisense Oligonucleotide Therapeutic Approach for Suppression of Ataxin-1 Expression: A Safety Assessment
Brennon L. O'Callaghan, Bente Hofstra, Hillary P. Handler, Holly B. Kordasiewicz +11 more
2020Molecular Therapy: Nucleic Acids22 citations Open access
Semantic Scholar
Glycogen synthase downregulation rescues the amylopectinosis of murine RBCK1 deficiency.
S. Nitschke, Mitchell A. Sullivan, Sharmistha Mitra, Charlotte R. Marchioni +17 more
2022Brain : a journal of neurology21 citations Open access
Semantic Scholar
Neural deficits in a mouse model of PACS1 syndrome are corrected with PACS1- or HDAC6-targeting therapy
Sabrina Villar-Pazos, Laurel Thomas, Yunhan Yang, Kun Chen +10 more
2023Nature Communications20 citations Open access
Semantic Scholar
A single-cell map of antisense oligonucleotide activity in the brain
M. Mortberg, Juliana E. Gentile, Naeem M. Nadaf, Charles R. Vanderburg +10 more
2023bioRxiv18 citations Open access
Semantic Scholar

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