Harvard Catalyst Profiles

Contact, publication, and social network information about Harvard faculty and fellows.

Vanessa Chantal Wheeler, Ph.D.


The research activities and funding listed below are automatically derived from NIH ExPORTER and other sources, which might result in incorrect or missing items. Faculty can login to make corrections and additions.
  1. R21NS111066 (WHEELER, VANESSA C) Apr 1, 2019 - Mar 31, 2021
    Huntington's disease gene chromatin structure and modifiers
    Role: Principal Investigator
  2. R21NS109755 (WHEELER, VANESSA C) Feb 15, 2019 - Jan 31, 2021
    Modifiers of C9orf72 repeat dynamics
    Role: Principal Investigator
  3. R01NS049206 (WHEELER, VANESSA C) Jul 1, 2004 - Jun 30, 2021
    Huntington's disease repeat instability and pathogenesis
    Role: Principal Investigator

Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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  1. Lee JM, Huang Y, Orth M, Gillis T, Siciliano J, Hong E, Mysore JS, Lucente D, Wheeler VC, Seong IS, McLean ZL, Mills JA, McAllister B, Lobanov SV, Massey TH, Ciosi M, Landwehrmeyer GB, Paulsen JS, Dorsey ER, Shoulson I, Sampaio C, Monckton DG, Kwak S, Holmans P, Jones L, MacDonald ME, Long JD, Gusella JF. Genetic modifiers of Huntington disease differentially influence motor and cognitive domains. Am J Hum Genet. 2022 Mar 18. PMID: 35325614.
    Citations:    Fields:    
  2. Roy JCL, Vitalo A, Andrew MA, Mota-Silva E, Kovalenko M, Burch Z, Nhu AM, Cohen PE, Grabczyk E, Wheeler VC, Mouro Pinto R. Somatic CAG expansion in Huntington's disease is dependent on the MLH3 endonuclease domain, which can be excluded via splice redirection. Nucleic Acids Res. 2021 04 19; 49(7):3907-3918. PMID: 33751106.
    Citations:    Fields:    Translation:HumansAnimalsCells
  3. Hong EP, Chao MJ, Massey T, McAllister B, Lobanov S, Jones L, Holmans P, Kwak S, Orth M, Ciosi M, Monckton DG, Long JD, Lucente D, Wheeler VC, MacDonald ME, Gusella JF, Lee JM. Association Analysis of Chromosome X to Identify Genetic Modifiers of Huntington's Disease. J Huntingtons Dis. 2021; 10(3):367-375. PMID: 34180418.
    Citations:    Fields:    Translation:HumansCells
  4. Jones L, Wheeler VC, Pearson CE. Special Issue: DNA Repair and Somatic Repeat Expansion in Huntington's Disease. J Huntingtons Dis. 2021; 10(1):3-5. PMID: 33554921.
    Citations:    Fields:    Translation:HumansAnimalsCells
  5. Hong EP, MacDonald ME, Wheeler VC, Jones L, Holmans P, Orth M, Monckton DG, Long JD, Kwak S, Gusella JF, Lee JM. Huntington's Disease Pathogenesis: Two Sequential Components. J Huntingtons Dis. 2021; 10(1):35-51. PMID: 33579862.
    Citations: 12     Fields:    Translation:HumansCells
  6. Ciosi M, Cumming SA, Chatzi A, Larson E, Tottey W, Lomeikaite V, Hamilton G, Wheeler VC, Pinto RM, Kwak S, Morton AJ, Monckton DG. Approaches to Sequence the HTT CAG Repeat Expansion and Quantify Repeat Length Variation. J Huntingtons Dis. 2021; 10(1):53-74. PMID: 33579864.
    Citations: 5     Fields:    Translation:AnimalsCells
  7. Wheeler VC, Dion V. Modifiers of CAG/CTG Repeat Instability: Insights from Mammalian Models. J Huntingtons Dis. 2021; 10(1):123-148. PMID: 33579861.
    Citations: 12     Fields:    Translation:AnimalsCells
  8. Loupe JM, Pinto RM, Kim KH, Gillis T, Mysore JS, Andrew MA, Kovalenko M, Murtha R, Seong I, Gusella JF, Kwak S, Howland D, Lee R, Lee JM, Wheeler VC, MacDonald ME. Promotion of somatic CAG repeat expansion by Fan1 knock-out in Huntington's disease knock-in mice is blocked by Mlh1 knock-out. Hum Mol Genet. 2020 11 04; 29(18):3044-3053. PMID: 32876667.
    Citations: 16     Fields:    Translation:HumansAnimalsCells
  9. Kovalenko M, Erdin S, Andrew MA, St Claire J, Shaughnessey M, Hubert L, Neto JL, Stortchevoi A, Fass DM, Mouro Pinto R, Haggarty SJ, Wilson JH, Talkowski ME, Wheeler VC. Histone deacetylase knockouts modify transcription, CAG instability and nuclear pathology in Huntington disease mice. Elife. 2020 09 29; 9. PMID: 32990597.
    Citations: 5     Fields:    Translation:AnimalsCells
  10. Mouro Pinto R, Arning L, Giordano JV, Razghandi P, Andrew MA, Gillis T, Correia K, Mysore JS, Grote Urtubey DM, Parwez CR, von Hein SM, Clark HB, Nguyen HP, Förster E, Beller A, Jayadaev S, Keene CD, Bird TD, Lucente D, Vonsattel JP, Orr H, Saft C, Petrasch-Parwez E, Wheeler VC. Patterns of CAG repeat instability in the central nervous system and periphery in Huntington's disease and in spinocerebellar ataxia type 1. Hum Mol Genet. 2020 08 29; 29(15):2551-2567. PMID: 32761094.
    Citations: 16     Fields:    Translation:HumansCells
  11. Ellis N, Tee A, McAllister B, Massey T, McLauchlan D, Stone T, Correia K, Loupe J, Kim KH, Barker D, Hong EP, Chao MJ, Long JD, Lucente D, Vonsattel JPG, Pinto RM, Elneel KA, Ramos EM, Mysore JS, Gillis T, Wheeler VC, Medway C, Hall L, Kwak S, Sampaio C, Ciosi M, Maxwell A, Chatzi A, Monckton DG, Orth M, Landwehrmeyer GB, Paulsen JS, Shoulson I, Myers RH, van Duijn E, Rickards H, MacDonald ME, Lee JM, Gusella JF, Jones L, Holmans P. Genetic Risk Underlying Psychiatric and Cognitive Symptoms in Huntington's Disease. Biol Psychiatry. 2020 05 01; 87(9):857-865. PMID: 32087949.
    Citations: 6     Fields:    Translation:Humans
  12. Aviolat H, Pinto RM, Godschall E, Murtha R, Richey HE, Sapp E, Vodicka P, Wheeler VC, Kegel-Gleason KB, DiFiglia M. Assessing average somatic CAG repeat instability at the protein level. Sci Rep. 2019 12 16; 9(1):19152. PMID: 31844074.
    Citations: 5     Fields:    Translation:HumansAnimalsCells
  13. Hung CL, Maiuri T, Bowie LE, Gotesman R, Son S, Falcone M, Giordano JV, Gillis T, Mattis V, Lau T, Kwan V, Wheeler V, Schertzer J, Singh K, Truant R. A patient-derived cellular model for Huntington's disease reveals phenotypes at clinically relevant CAG lengths. Mol Biol Cell. 2018 11 15; 29(23):2809-2820. PMID: 30256717.
    Citations: 7     Fields:    Translation:HumansCells
  14. Chao MJ, Kim KH, Shin JW, Lucente D, Wheeler VC, Li H, Roach JC, Hood L, Wexler NS, Jardim LB, Holmans P, Jones L, Orth M, Kwak S, MacDonald ME, Gusella JF, Lee JM. Population-specific genetic modification of Huntington's disease in Venezuela. PLoS Genet. 2018 05; 14(5):e1007274. PMID: 29750799.
    Citations: 10     Fields:    Translation:Humans
  15. Ament SA, Pearl JR, Cantle JP, Bragg RM, Skene PJ, Coffey SR, Bergey DE, Wheeler VC, MacDonald ME, Baliga NS, Rosinski J, Hood LE, Carroll JB, Price ND. Transcriptional regulatory networks underlying gene expression changes in Huntington's disease. Mol Syst Biol. 2018 03 26; 14(3):e7435. PMID: 29581148.
    Citations: 20     Fields:    Translation:HumansAnimals
  16. Kovalenko M, Milnerwood A, Giordano J, St Claire J, Guide JR, Stromberg M, Gillis T, Sapp E, DiFiglia M, MacDonald ME, Carroll JB, Lee JM, Tappan S, Raymond L, Wheeler VC. HttQ111/+ Huntington's Disease Knock-in Mice Exhibit Brain Region-Specific Morphological Changes and Synaptic Dysfunction. J Huntingtons Dis. 2018; 7(1):17-33. PMID: 29480209.
    Citations: 8     Fields:    Translation:AnimalsCells
  17. Lee JM, Chao MJ, Harold D, Abu Elneel K, Gillis T, Holmans P, Jones L, Orth M, Myers RH, Kwak S, Wheeler VC, MacDonald ME, Gusella JF. A modifier of Huntington's disease onset at the MLH1 locus. Hum Mol Genet. 2017 10 01; 26(19):3859-3867. PMID: 28934397.
    Citations: 30     Fields:    Translation:HumansAnimalsCells
  18. Azevedo A, Kovalenko M, Andrew M, Zhang F, Lee J, Wheeler V, Pinto MR. Identification of genetic modifiers of somatic CAG instability in Huntington's Disease by in vivo CRISPR - Cas9 genome editing: PS238. Porto Biomed J. 2017 Sep-Oct; 2(5):209-210. PMID: 32258693.
  19. Chao MJ, Gillis T, Atwal RS, Mysore JS, Arjomand J, Harold D, Holmans P, Jones L, Orth M, Myers RH, Kwak S, Wheeler VC, MacDonald ME, Gusella JF, Lee JM. Haplotype-based stratification of Huntington's disease. Eur J Hum Genet. 2017 11; 25(11):1202-1209. PMID: 28832564.
    Citations: 12     Fields:    Translation:HumansCells
  20. Gasset-Rosa F, Chillon-Marinas C, Goginashvili A, Atwal RS, Artates JW, Tabet R, Wheeler VC, Bang AG, Cleveland DW, Lagier-Tourenne C. Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport. Neuron. 2017 Apr 05; 94(1):48-57.e4. PMID: 28384474.
    Citations: 78     Fields:    Translation:HumansAnimalsCells
  21. Bragg RM, Coffey SR, Weston RM, Ament SA, Cantle JP, Minnig S, Funk CC, Shuttleworth DD, Woods EL, Sullivan BR, Jones L, Glickenhaus A, Anderson JS, Anderson MD, Dunnett SB, Wheeler VC, MacDonald ME, Brooks SP, Price ND, Carroll JB. Corrigendum: Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington's disease. Sci Rep. 2017 03 28; 7:44960. PMID: 28350386.
    Citations:    Fields:    
  22. Ament SA, Pearl JR, Grindeland A, St Claire J, Earls JC, Kovalenko M, Gillis T, Mysore J, Gusella JF, Lee JM, Kwak S, Howland D, Lee MY, Baxter D, Scherler K, Wang K, Geman D, Carroll JB, MacDonald ME, Carlson G, Wheeler VC, Price ND, Hood LE. High resolution time-course mapping of early transcriptomic, molecular and cellular phenotypes in Huntington's disease CAG knock-in mice across multiple genetic backgrounds. Hum Mol Genet. 2017 03 01; 26(5):913-922. PMID: 28334820.
    Citations: 16     Fields:    Translation:HumansAnimalsCells
  23. Bragg RM, Coffey SR, Weston RM, Ament SA, Cantle JP, Minnig S, Funk CC, Shuttleworth DD, Woods EL, Sullivan BR, Jones L, Glickenhaus A, Anderson JS, Anderson MD, Dunnett SB, Wheeler VC, MacDonald ME, Brooks SP, Price ND, Carroll JB. Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington's disease. Sci Rep. 2017 02 08; 7:41570. PMID: 28176805.
    Citations: 5     Fields:    
  24. Neto JL, Lee JM, Afridi A, Gillis T, Guide JR, Dempsey S, Lager B, Alonso I, Wheeler VC, Pinto RM. Genetic Contributors to Intergenerational CAG Repeat Instability in Huntington's Disease Knock-In Mice. Genetics. 2017 02; 205(2):503-516. PMID: 27913616.
    Citations: 10     Fields:    Translation:AnimalsCells
  25. Alexandrov V, Brunner D, Menalled LB, Kudwa A, Watson-Johnson J, Mazzella M, Russell I, Ruiz MC, Torello J, Sabath E, Sanchez A, Gomez M, Filipov I, Cox K, Kwan M, Ghavami A, Ramboz S, Lager B, Wheeler VC, Aaronson J, Rosinski J, Gusella JF, MacDonald ME, Howland D, Kwak S. Large-scale phenome analysis defines a behavioral signature for Huntington's disease genotype in mice. Nat Biotechnol. 2016 08; 34(8):838-44. PMID: 27376585.
    Citations: 20     Fields:    Translation:Animals
  26. Ramos EM, Kovalenko M, Guide JR, St Claire J, Gillis T, Mysore JS, Sequeiros J, Wheeler VC, Alonso I, MacDonald ME. Chromosome substitution strain assessment of a Huntington's disease modifier locus. Mamm Genome. 2015 Apr; 26(3-4):119-30. PMID: 25645993.
    Citations: 3     Fields:    Translation:HumansAnimalsCells
  27. Correia K, Harold D, Kim KH, Holmans P, Jones L, Orth M, Myers RH, Kwak S, Wheeler VC, MacDonald ME, Gusella JF, Lee JM. The Genetic Modifiers of Motor OnsetAge (GeM MOA) Website: Genome-wide Association Analysis for Genetic Modifiers of Huntington's Disease. J Huntingtons Dis. 2015; 4(3):279-84. PMID: 26444025.
    Citations: 14     Fields:    Translation:Humans
  28. Blumenthal I, Ragavendran A, Erdin S, Klei L, Sugathan A, Guide JR, Manavalan P, Zhou JQ, Wheeler VC, Levin JZ, Ernst C, Roeder K, Devlin B, Gusella JF, Talkowski ME. Transcriptional consequences of 16p11.2 deletion and duplication in mouse cortex and multiplex autism families. Am J Hum Genet. 2014 Jun 05; 94(6):870-83. PMID: 24906019.
    Citations: 56     Fields:    Translation:HumansAnimalsCells
  29. Galkina EI, Shin A, Coser KR, Shioda T, Kohane IS, Seong IS, Wheeler VC, Gusella JF, Macdonald ME, Lee JM. HD CAGnome: a search tool for huntingtin CAG repeat length-correlated genes. PLoS One. 2014; 9(4):e95556. PMID: 24751919.
    Citations: 1     Fields:    Translation:HumansCells
  30. Hölter SM, Stromberg M, Kovalenko M, Garrett L, Glasl L, Lopez E, Guide J, Götz A, Hans W, Becker L, Rathkolb B, Rozman J, Schrewed A, Klingenspor M, Klopstock T, Schulz H, Wolf E, Wursta W, Gillis T, Wakimoto H, Seidman J, MacDonald ME, Cotman S, Gailus-Durner V, Fuchs H, de Angelis MH, Lee JM, Wheeler VC. A broad phenotypic screen identifies novel phenotypes driven by a single mutant allele in Huntington's disease CAG knock-in mice. PLoS One. 2013; 8(11):e80923. PMID: 24278347.
    Citations: 20     Fields:    Translation:AnimalsCells
  31. Pinto RM, Dragileva E, Kirby A, Lloret A, Lopez E, St Claire J, Panigrahi GB, Hou C, Holloway K, Gillis T, Guide JR, Cohen PE, Li GM, Pearson CE, Daly MJ, Wheeler VC. Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches. PLoS Genet. 2013 Oct; 9(10):e1003930. PMID: 24204323.
    Citations: 90     Fields:    Translation:HumansAnimalsCells
  32. Lee JM, Galkina EI, Levantovsky RM, Fossale E, Anne Anderson M, Gillis T, Srinidhi Mysore J, Coser KR, Shioda T, Zhang B, Furia MD, Derry J, Kohane IS, Seong IS, Wheeler VC, Gusella JF, MacDonald ME. 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. Hum Mol Genet. 2013 Aug 15; 22(16):3227-38. PMID: 23595883.
    Citations: 16     Fields:    Translation:HumansCells
  33. Kovalenko M, Dragileva E, St Claire J, Gillis T, Guide JR, New J, Dong H, Kucherlapati R, Kucherlapati MH, Ehrlich ME, Lee JM, Wheeler VC. Msh2 acts in medium-spiny striatal neurons as an enhancer of CAG instability and mutant huntingtin phenotypes in Huntington's disease knock-in mice. PLoS One. 2012; 7(9):e44273. PMID: 22970194.
    Citations: 32     Fields:    Translation:AnimalsCells
  34. Staropoli JF, Haliw L, Biswas S, Garrett L, Hölter SM, Becker L, Skosyrski S, Da Silva-Buttkus P, Calzada-Wack J, Neff F, Rathkolb B, Rozman J, Schrewe A, Adler T, Puk O, Sun M, Favor J, Racz I, Bekeredjian R, Busch DH, Graw J, Klingenspor M, Klopstock T, Wolf E, Wurst W, Zimmer A, Lopez E, Harati H, Hill E, Krause DS, Guide J, Dragileva E, Gale E, Wheeler VC, Boustany RM, Brown DE, Breton S, Ruether K, Gailus-Durner V, Fuchs H, de Angelis MH, Cotman SL. Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous system. PLoS One. 2012; 7(6):e38310. PMID: 22701626.
    Citations: 30     Fields:    Translation:AnimalsCells
  35. Mochel F, Durant B, Meng X, O'Callaghan J, Yu H, Brouillet E, Wheeler VC, Humbert S, Schiffmann R, Durr A. Early alterations of brain cellular energy homeostasis in Huntington disease models. J Biol Chem. 2012 Jan 06; 287(2):1361-70. PMID: 22123819.
    Citations: 57     Fields:    Translation:Animals
  36. Lee JM, Pinto RM, Gillis T, St Claire JC, Wheeler VC. Quantification of age-dependent somatic CAG repeat instability in Hdh CAG knock-in mice reveals different expansion dynamics in striatum and liver. PLoS One. 2011; 6(8):e23647. PMID: 21897851.
    Citations: 32     Fields:    Translation:HumansAnimalsCells
  37. Fossale E, Seong IS, Coser KR, Shioda T, Kohane IS, Wheeler VC, Gusella JF, MacDonald ME, Lee JM. Differential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative. Hum Mol Genet. 2011 Nov 01; 20(21):4258-67. PMID: 21840924.
    Citations: 16     Fields:    Translation:AnimalsCells
  38. Lee JM, Zhang J, Su AI, Walker JR, Wiltshire T, Kang K, Dragileva E, Gillis T, Lopez ET, Boily MJ, Cyr M, Kohane I, Gusella JF, MacDonald ME, Wheeler VC. A novel approach to investigate tissue-specific trinucleotide repeat instability. BMC Syst Biol. 2010 Mar 19; 4:29. PMID: 20302627.
    Citations: 52     Fields:    Translation:AnimalsCells
  39. Goula AV, Berquist BR, Wilson DM, Wheeler VC, Trottier Y, Merienne K. Stoichiometry of base excision repair proteins correlates with increased somatic CAG instability in striatum over cerebellum in Huntington's disease transgenic mice. PLoS Genet. 2009 Dec; 5(12):e1000749. PMID: 19997493.
    Citations: 80     Fields:    Translation:AnimalsCells
  40. Seong IS, Woda JM, Song JJ, Lloret A, Abeyrathne PD, Woo CJ, Gregory G, Lee JM, Wheeler VC, Walz T, Kingston RE, Gusella JF, Conlon RA, MacDonald ME. Huntingtin facilitates polycomb repressive complex 2. Hum Mol Genet. 2010 Feb 15; 19(4):573-83. PMID: 19933700.
    Citations: 85     Fields:    Translation:HumansAnimalsCells
  41. Hendricks AE, Latourelle JC, Lunetta KL, Cupples LA, Wheeler V, MacDonald ME, Gusella JF, Myers RH. 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). Am J Med Genet A. 2009 Jul; 149A(7):1375-81. PMID: 19507258.
    Citations: 19     Fields:    Translation:HumansCells
  42. Swami M, Hendricks AE, Gillis T, Massood T, Mysore J, Myers RH, Wheeler VC. Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset. Hum Mol Genet. 2009 Aug 15; 18(16):3039-47. PMID: 19465745.
    Citations: 116     Fields:    Translation:HumansCells
  43. Menalled L, El-Khodor BF, Patry M, Suárez-Fariñas M, Orenstein SJ, Zahasky B, Leahy C, Wheeler V, Yang XW, MacDonald M, Morton AJ, Bates G, Leeds J, Park L, Howland D, Signer E, Tobin A, Brunner D. Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models. Neurobiol Dis. 2009 Sep; 35(3):319-36. PMID: 19464370.
    Citations: 144     Fields:    Translation:HumansAnimals
  44. Dragileva E, Hendricks A, Teed A, Gillis T, Lopez ET, Friedberg EC, Kucherlapati R, Edelmann W, Lunetta KL, MacDonald ME, Wheeler VC. Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes. Neurobiol Dis. 2009 Jan; 33(1):37-47. PMID: 18930147.
    Citations: 103     Fields:    Translation:AnimalsCells
  45. Wheeler VC, Persichetti F, McNeil SM, Mysore JS, Mysore SS, MacDonald ME, Myers RH, Gusella JF, Wexler NS. Factors associated with HD CAG repeat instability in Huntington disease. J Med Genet. 2007 Nov; 44(11):695-701. PMID: 17660463.
    Citations: 43     Fields:    Translation:HumansCells
  46. Li JL, Hayden MR, Warby SC, Durr A, Morrison PJ, Nance M, Ross CA, Margolis RL, Rosenblatt A, Squitieri F, Frati L, Gómez-Tortosa E, García CA, Suchowersky O, Klimek ML, Trent RJ, McCusker E, Novelletto A, Frontali M, Paulsen JS, Jones R, Ashizawa T, Lazzarini A, Wheeler VC, Prakash R, Xu G, Djoussé L, Mysore JS, Gillis T, Hakky M, Cupples LA, Saint-Hilaire MH, Cha JH, Hersch SM, Penney JB, Harrison MB, Perlman SL, Zanko A, Abramson RK, Lechich AJ, Duckett A, Marder K, Conneally PM, Gusella JF, MacDonald ME, Myers RH. Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study. BMC Med Genet. 2006 Aug 17; 7:71. PMID: 16914060.
    Citations: 34     Fields:    Translation:HumansCells
  47. Guidetti P, Bates GP, Graham RK, Hayden MR, Leavitt BR, MacDonald ME, Slow EJ, Wheeler VC, Woodman B, Schwarcz R. Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice. Neurobiol Dis. 2006 Jul; 23(1):190-7. PMID: 16697652.
    Citations: 54     Fields:    Translation:HumansAnimals
  48. Lloret A, Dragileva E, Teed A, Espinola J, Fossale E, Gillis T, Lopez E, Myers RH, MacDonald ME, Wheeler VC. Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice. Hum Mol Genet. 2006 Jun 15; 15(12):2015-24. PMID: 16687439.
    Citations: 48     Fields:    Translation:AnimalsCells
  49. Li JL, Hayden MR, Almqvist EW, Brinkman RR, Durr A, Dodé C, Morrison PJ, Suchowersky O, Ross CA, Margolis RL, Rosenblatt A, Gómez-Tortosa E, Cabrero DM, Novelletto A, Frontali M, Nance M, Trent RJ, McCusker E, Jones R, Paulsen JS, Harrison M, Zanko A, Abramson RK, Russ AL, Knowlton B, Djoussé L, Mysore JS, Tariot S, Gusella MF, Wheeler VC, Atwood LD, Cupples LA, Saint-Hilaire M, Cha JH, Hersch SM, Koroshetz WJ, Gusella JF, MacDonald ME, Myers RH. A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study. Am J Hum Genet. 2003 Sep; 73(3):682-7. PMID: 12900792.
    Citations: 58     Fields:    Translation:HumansCells
  50. Gines S, Seong IS, Fossale E, Ivanova E, Trettel F, Gusella JF, Wheeler VC, Persichetti F, MacDonald ME. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet. 2003 Mar 01; 12(5):497-508. PMID: 12588797.
    Citations: 115     Fields:    Translation:Animals
  51. Wheeler VC, Lebel LA, Vrbanac V, Teed A, te Riele H, MacDonald ME. Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum. Hum Mol Genet. 2003 Feb 01; 12(3):273-81. PMID: 12554681.
    Citations: 108     Fields:    Translation:HumansAnimalsCells
  52. Snider BJ, Moss JL, Revilla FJ, Lee CS, Wheeler VC, Macdonald ME, Choi DW. Neocortical neurons cultured from mice with expanded CAG repeats in the huntingtin gene: unaltered vulnerability to excitotoxins and other insults. Neuroscience. 2003; 120(3):617-25. PMID: 12895502.
    Citations: 3     Fields:    Translation:AnimalsCells
  53. MacDonald ME, Gines S, Gusella JF, Wheeler VC. Huntington's disease. Neuromolecular Med. 2003; 4(1-2):7-20. PMID: 14528049.
    Citations: 30     Fields:    Translation:HumansAnimalsCells
  54. Namura S, Hirt L, Wheeler VC, McGinnis KM, Hilditch-Maguire P, Moskowitz MA, MacDonald ME, Persichetti F. The HD mutation does not alter neuronal death in the striatum of Hdh(Q92) knock-in mice after mild focal ischemia. Neurobiol Dis. 2002 Oct; 11(1):147-54. PMID: 12460554.
    Citations: 2     Fields:    Translation:AnimalsCells
  55. Fossale E, Wheeler VC, Vrbanac V, Lebel LA, Teed A, Mysore JS, Gusella JF, MacDonald ME, Persichetti F. Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice. Hum Mol Genet. 2002 Sep 15; 11(19):2233-41. PMID: 12217951.
    Citations: 27     Fields:    Translation:HumansAnimalsCells
  56. Wheeler VC, Gutekunst CA, Vrbanac V, Lebel LA, Schilling G, Hersch S, Friedlander RM, Gusella JF, Vonsattel JP, Borchelt DR, MacDonald ME. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet. 2002 Mar 15; 11(6):633-40. PMID: 11912178.
    Citations: 75     Fields:    Translation:AnimalsCells
  57. Auerbach W, Hurlbert MS, Hilditch-Maguire P, Wadghiri YZ, Wheeler VC, Cohen SI, Joyner AL, MacDonald ME, Turnbull DH. The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin. Hum Mol Genet. 2001 Oct 15; 10(22):2515-23. PMID: 11709539.
    Citations: 50     Fields:    Translation:Animals
  58. Trettel F, Rigamonti D, Hilditch-Maguire P, Wheeler VC, Sharp AH, Persichetti F, Cattaneo E, MacDonald ME. Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells. Hum Mol Genet. 2000 Nov 22; 9(19):2799-809. PMID: 11092756.
    Citations: 293     Fields:    Translation:HumansAnimalsCells
  59. Wheeler VC, White JK, Gutekunst CA, Vrbanac V, Weaver M, Li XJ, Li SH, Yi H, Vonsattel JP, Gusella JF, Hersch S, Auerbach W, Joyner AL, MacDonald ME. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet. 2000 Mar 01; 9(4):503-13. PMID: 10699173.
    Citations: 157     Fields:    Translation:AnimalsCells
  60. Wheeler VC, Auerbach W, White JK, Srinidhi J, Auerbach A, Ryan A, Duyao MP, Vrbanac V, Weaver M, Gusella JF, Joyner AL, MacDonald ME. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet. 1999 Jan; 8(1):115-22. PMID: 9887339.
    Citations: 140     Fields:    Translation:HumansAnimalsCells
  61. Wheeler VC, Aitken M, Coutelle C. Modification of the mouse mitochondrial genome by insertion of an exogenous gene. Gene. 1997 Oct 01; 198(1-2):203-9. PMID: 9370282.
    Citations: 5     Fields:    Translation:HumansAnimals
  62. Collombet JM, Wheeler VC, Vogel F, Coutelle C. Introduction of plasmid DNA into isolated mitochondria by electroporation. A novel approach toward gene correction for mitochondrial disorders. J Biol Chem. 1997 Feb 21; 272(8):5342-7. PMID: 9030609.
    Citations: 29     Fields:    Translation:AnimalsCells
  63. Wheeler VC, Prodromou C, Pearl LH, Williamson R, Coutelle C. Synthesis of a modified gene encoding human ornithine transcarbamylase for expression in mammalian mitochondrial and universal translation systems: a novel approach towards correction of a genetic defect. Gene. 1996 Mar 09; 169(2):251-5. PMID: 8647457.
    Citations: 5     Fields:    Translation:HumansAnimalsCells
  64. Wheeler VC, Coutelle C. Nondegradative in vitro labeling of plasmid DNA. Anal Biochem. 1995 Mar 01; 225(2):374-6. PMID: 7762810.
    Citations:    Fields:    Translation:Cells
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Funded by the NIH National Center for Advancing Translational Sciences through its Clinical and Translational Science Awards Program, grant number UL1TR002541.