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V. Wheeler · SciRah

Researcher profile · Semantic Scholar

V. Wheeler

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V. Wheeler — researcher profile on SciRah

h-index: 42
Works: 119
Citations: 8,557

Most-cited papers

Identification of Genetic Factors that Modify Clinical Onset of Huntington’s Disease
Jong-Min Lee, V. Wheeler, M. Chao, J. Vonsattel +23 more
2015Cell563 citations Open access
Semantic Scholar
Induced Pluripotent Stem Cells from Patients with Huntington’s Disease Show CAG Repeat Expansion Associated Phenotypes
Virginia B. Mattis, Soshana P. Svendsen, A. Ebert, C. Svendsen +54 more
2012Cell Stem Cell473 citations Open access
Semantic Scholar
Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset.
M. Swami, A. Hendricks, T. Gillis, T. Massood +3 more
2009Human Molecular Genetics350 citations Open access
Semantic Scholar
Systematic behavioral evaluation of Huntington’s disease transgenic and knock-in mouse models
L. Menalled, Bassem F. El-Khodor, Monica Patry, M. Suárez-Fariñas +14 more
2009Neurobiology of Disease342 citations Open access
Semantic Scholar
CAG Repeat Not Polyglutamine Length Determines Timing of Huntington’s Disease Onset
Jong-Min Lee, Kevin Correia, Jacob M. Loupe, Kyung-Hee Kim +34 more
2019Cell300 citations Open access
Semantic Scholar
Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes
E. Dragileva, A. Hendricks, Allison M Teed, T. Gillis +7 more
2008Neurobiology of Disease250 citations Open access
Semantic Scholar
Mismatch Repair Genes Mlh1 and Mlh3 Modify CAG Instability in Huntington's Disease Mice: Genome-Wide and Candidate Approaches
R. M. Pinto, E. Dragileva, Andrew W. Kirby, Alejandro Lloret +12 more
2013PLoS Genetics244 citations Open access
Semantic Scholar
Polyglutamine-expanded huntingtin exacerbates age-related disruption of nuclear integrity and nucleocytoplasmic transport
Fátima Gasset‐Rosa, Carlos Chillón-Marinas, A. Goginashvili, R. Atwal +6 more
2017Neuron221 citations Open access
Semantic Scholar
Huntingtin facilitates polycomb repressive complex 2
I. Seong, J. Woda, Ji-Joon Song, Alejandro Lloret +10 more
2009Human Molecular Genetics180 citations Open access
Semantic Scholar
A novel approach to investigate tissue-specific trinucleotide repeat instability
Jong-Min Lee, Jie Zhang, Andrew I. Su, J. Walker +11 more
2010BMC Systems Biology148 citations Open access
Semantic Scholar
Stoichiometry of Base Excision Repair Proteins Correlates with Increased Somatic CAG Instability in Striatum over Cerebellum in Huntington's Disease Transgenic Mice
Agathi-Vasiliki Goula, Brian R. Berquist, D. Wilson, V. Wheeler +2 more
2009PLoS Genetics146 citations Open access
Semantic Scholar
Factors associated with HD CAG repeat instability in Huntington disease
V. Wheeler, F. Persichetti, S. McNeil, J. Mysore +5 more
2007Journal of Medical Genetics146 citations Open access
Semantic Scholar
Early Alterations of Brain Cellular Energy Homeostasis in Huntington Disease Models*
F. Mochel, Brandon Durant, Xi-Na Meng, James P. O'Callaghan +6 more
2011Journal of Biological Chemistry132 citations Open access
Semantic Scholar
Transcriptional consequences of 16p11.2 deletion and duplication in mouse cortex and multiplex autism families.
Ian Blumenthal, A. Ragavendran, S. Erdin, L. Klei +11 more
2014American Journal of Human Genetics128 citations Open access
Semantic Scholar
A modifier of Huntington's disease onset at the MLH1 locus
Jong-Min Lee, M. Chao, D. Harold, Kawther Abu Elneel +9 more
2017Human Molecular Genetics106 citations Open access
Semantic Scholar
Msh2 Acts in Medium-Spiny Striatal Neurons as an Enhancer of CAG Instability and Mutant Huntingtin Phenotypes in Huntington’s Disease Knock-In Mice
Marina Kovalenko, E. Dragileva, Jason St. Claire, T. Gillis +8 more
2012PLoS ONE94 citations Open access
Semantic Scholar
Huntington’s Disease Pathogenesis: Two Sequential Components
E. Hong, M. MacDonald, V. Wheeler, L. Jones +7 more
2021Journal of Huntington's Disease89 citations Open access
Semantic Scholar
Quantification of Age-Dependent Somatic CAG Repeat Instability in Hdh CAG Knock-In Mice Reveals Different Expansion Dynamics in Striatum and Liver
Jong-Min Lee, R. M. Pinto, T. Gillis, Jason St. Claire +1 more
2011PLoS ONE85 citations Open access
Semantic Scholar
Genetic modifiers of Huntington disease differentially influence motor and cognitive domains.
Jong-Min Lee, Yuan Huang, Michael Orth, T. Gillis +24 more
2022American Journal of Human Genetics83 citations Open access
Semantic Scholar
Promotion of somatic CAG repeat expansion by Fan1 knock-out in Huntington’s disease knock-in mice is blocked by Mlh1 knock-out
Jacob M. Loupe, R. M. Pinto, Kyung-Hee Kim, T. Gillis +12 more
2020Human Molecular Genetics74 citations Open access
Semantic Scholar
Exome sequencing of individuals with Huntington’s disease implicates FAN1 nuclease activity in slowing CAG expansion and disease onset
Branduff McAllister, Jasmine Donaldson, Caroline S. Binda, Sophie Powell +28 more
2022Nature Neuroscience72 citations Open access
Semantic Scholar
Transcriptional regulatory networks underlying gene expression changes in Huntington's disease
S. Ament, Jocelynn R. Pearl, J. P. Cantle, Robert M. Bragg +10 more
2018Molecular Systems Biology61 citations Open access
Semantic Scholar
Amplification-free, CRISPR-Cas9 Targeted Enrichment and SMRT Sequencing of Repeat-Expansion Disease Causative Genomic Regions
Yu-Chih Tsai, D. Greenberg, James Powell, Ida Höijer +11 more
2017bioRxiv61 citations Open access
Semantic Scholar
Large-Scale Phenotyping of an Accurate Genetic Mouse Model of JNCL Identifies Novel Early Pathology Outside the Central Nervous System
J. Staropoli, Larissa Haliw, Sunita Biswas, L. Garrett +38 more
2012PLoS ONE60 citations Open access
Semantic Scholar
Modifiers of CAG/CTG Repeat Instability: Insights from Mammalian Models
V. Wheeler, V. Dion
2021Journal of Huntington's Disease58 citations Open access
Semantic Scholar
In vivo CRISPR-Cas9 genome editing in mice identifies genetic modifiers of somatic CAG repeat instability in Huntington’s disease
R. M. Pinto, Ryan Murtha, A. Azevedo, Cameron Douglas +18 more
2025Nature Genetics51 citations Open access
Semantic Scholar
Estimating the Probability of de novo HD cases from Transmissions of Expanded Penetrant CAG Alleles in the Huntington Disease Gene from Male Carriers of High Normal Alleles (27–35 CAG)
A. Hendricks, J. Latourelle, Kathryn L. Lunetta, L. Cupples +4 more
2009American Journal of Medical Genetics. Part A50 citations Open access
Semantic Scholar
Large-scale phenome analysis defines a behavioral signature for Huntington's disease genotype in mice
Vadim Alexandrov, D. Brunner, L. Menalled, A. Kudwa +21 more
2016Nature Biotechnology49 citations
Semantic Scholar
Genetic modifiers of somatic expansion and clinical phenotypes in Huntington’s disease highlight shared and tissue-specific effects
Jong-Min Lee, Zachariah L McLean, Kevin Correia, Jun-Wan Shin +34 more
2025Nature Genetics44 citations Open access
Semantic Scholar
Population-specific genetic modification of Huntington's disease in Venezuela
M. Chao, Kyung-Hee Kim, Jun-Wan Shin, Diane E. Lucente +13 more
2018PLoS Genetics43 citations Open access
Semantic Scholar
A patient-derived cellular model for Huntington’s disease reveals phenotypes at clinically relevant CAG lengths
C. Hung, Tamara Maiuri, Laura E. Bowie, R. Gotesman +11 more
2018bioRxiv41 citations Open access
Semantic Scholar
A Broad Phenotypic Screen Identifies Novel Phenotypes Driven by a Single Mutant Allele in Huntington’s Disease CAG Knock-In Mice
S. Hölter, M. Stromberg, Marina Kovalenko, L. Garrett +24 more
2013PLoS ONE41 citations Open access
Semantic Scholar
Genetic Risk Underlying Psychiatric and Cognitive Symptoms in Huntington’s Disease
N. Ellis, Amelia Tee, Branduff McAllister, Thomas H. Massey +37 more
2019bioRxiv40 citations Open access
Semantic Scholar
High resolution time‐course mapping of early transcriptomic, molecular and cellular phenotypes in Huntington's disease CAG knock‐in mice across multiple genetic backgrounds
S. Ament, Jocelynn R. Pearl, Andrea Grindeland, Jason St. Claire +19 more
2017Human Molecular Genetics40 citations Open access
Semantic Scholar
Splice modulators target PMS1 to reduce somatic expansion of the Huntington’s disease-associated CAG repeat
Zachariah L McLean, D. Gao, Kevin Correia, Jennie C. L. Roy +15 more
2024Nature Communications37 citations Open access
Semantic Scholar
The Genetic Modifiers of Motor Onset Age (GeM MOA) website: genome-wide association analysis for genetic modifiers of Huntington's disease
Kevin Correia, D. Harold, Kyung-Hee Kim, P. Holmans +8 more
2015Journal of Huntington's Disease36 citations Open access
Semantic Scholar
Somatic CAG expansion in Huntington's disease is dependent on the MLH3 endonuclease domain, which can be excluded via splice redirection
Jennie C. L. Roy, A. Vitalo, M. Andrew, Eduarda Mota-Silva +7 more
2021Nucleic Acids Research35 citations Open access
Semantic Scholar
Huntington’s Disease
M. MacDonald, S. Ginés, J. Gusella, V. Wheeler
2007Neuromolecular medicine34 citations
Semantic Scholar
Approaches to Sequence the HTT CAG Repeat Expansion and Quantify Repeat Length Variation
Marc Ciosi, S. Cumming, A. Chatzi, Eloise Larson +8 more
2021Journal of Huntington's Disease30 citations Open access
Semantic Scholar
HttQ111/+ Huntington’s Disease Knock-in Mice Exhibit Brain Region-Specific Morphological Changes and Synaptic Dysfunction
Marina Kovalenko, A. Milnerwood, J. Giordano, Jason St. Claire +11 more
2018Journal of Huntington's Disease29 citations Open access
Semantic Scholar
Differential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative.
E. Fossale, I. Seong, Kathryn Coser, Toshi Shioda +5 more
2011Human Molecular Genetics29 citations Open access
Semantic Scholar
Genetic Contributors to Intergenerational CAG Repeat Instability in Huntington’s Disease Knock-In Mice
João Luís, Jong-Min Lee, Ali Afridi, T. Gillis +6 more
2016Genetics26 citations Open access
Semantic Scholar
Dominant effects of the Huntington's disease HTT CAG repeat length are captured in gene-expression data sets by a continuous analysis mathematical modeling strategy.
Jong-Min Lee, E. Galkina, Rachel M. Levantovsky, E. Fossale +13 more
2013Human Molecular Genetics25 citations Open access
Semantic Scholar
Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington’s disease
Robert M. Bragg, S. Coffey, R. Weston, S. Ament +16 more
2016Scientific Reports23 citations Open access
Semantic Scholar
Haplotype-based stratification of Huntington's disease
M. Chao, T. Gillis, R. Atwal, J. Mysore +11 more
2017European Journal of Human Genetics22 citations Open access
Semantic Scholar
CAG repeat expansion in the Huntington’s disease gene shapes linear and circular RNAs biogenesis
D. Ayyildiz, G. Bergonzoni, A. Monziani, T. Tripathi +13 more
2023PLoS Genetics21 citations Open access
Semantic Scholar
Assessing average somatic CAG repeat instability at the protein level
Hubert Aviolat, R. M. Pinto, Elizabeth N. Godschall, Ryan Murtha +6 more
2019Scientific Reports21 citations Open access
Semantic Scholar
Base editing strategies to convert CAG to CAA diminish the disease-causing mutation in Huntington's disease
Doo Eun Choi, Jun-Wan Shin, Sophia Zeng, E. Hong +6 more
2023bioRxiv19 citations Open access
Semantic Scholar
Tissue-specific and repeat length-dependent somatic instability of the X-linked dystonia parkinsonism-associated CCCTCT repeat
Lindsey N. Campion, Alan Meijia Maza, Rachita Yadav, E. Penney +21 more
2022Acta Neuropathologica Communications16 citations Open access
Semantic Scholar
Histone deacetylase knockouts modify transcription, CAG instability and nuclear pathology in Huntington disease mice
Marina Kovalenko, S. Erdin, M. Andrew, Jason St. Claire +10 more
2020eLife15 citations Open access
Semantic Scholar

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M. MacDonald×26
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Jong-Min Lee×24
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T. Gillis×24
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Seung Kwak — researcher profile on SciRah

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Diane E. Lucente — researcher profile on SciRah

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P. Holmans — researcher profile on SciRah

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L. Jones — researcher profile on SciRah

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M. Chao — researcher profile on SciRah