Faculty > Vanessa Wheeler    
       

Vanessa Wheeler, Ph.D.

Selected Publications

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;8:115-22.

 
OVERVIEW
 
LAB MEMBERS
 
PUBLICATIONS
 

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;9:503-513.

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;11:633-40.

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;15:2233-41.

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;12: 273-81.

Lloret A, Dragileva E, Teed A, Espinola J, Fossale E, Lopez E, Myers RH,
MacDonald ME and Wheeler VC. Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice. Hum. Mol. Genet. 2006:15 :2015-24.

Wheeler VC , Persichetti F , McNeil SM, Mysore JS, Mysore SS, MacDonald ME, Myers RH , Gusella JF, Wexler NS, The U.S-Venezuela Collaborative Research Group. Factors Associated with HD CAG repeat instability in Huntington's disease. J.Med Genet.2007; 44: 695-701

Dragileva E, Hendricks A, Teed A, Gillis T, Lopez E, Friedberg E,
Kucherlapati R, Edelmann W, Lunetta K, Macdonald ME and Wheeler VC.
Intergenerational and striatal CAG repeat instability in Huntington's
disease knock-in mice involve different DNA repair genes. Neurobiol.
Dis.2009; 33: 37-47.

Swami M, Hendricks AE, Gillis T, Massood T, Mysore J, Myers RH and Wheeler VC (2009). 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; 18(16):3039-47.

Menalled L, El-Khodor BF, Patry M, Suarez-Farinas M, Orestein S, Zahasky B,
Leahy C, Wheeler VC, Yang W, MacDonald ME, Morton J, Bates G, Leeds J, Park L, Howland D, Signer D, Tobin A and Brunner D. Systematic behavioral
evaluation of Huntington's disease transgenic and knock-in mouse models.
Neurobiol. Dis.2009; 35(3):319-36.

Hendricks AE, Latourelle JC, Lunetta KL, Cupples A, Wheeler VC, MacDonald
ME, Gusella JF, Myers, RH. Estimating the probability of de novo HD cases
from transmissions of expanded CAG alleles in the Huntington's disease gene from male carriers of high normal alleles (27-35 CAG). Am J Med Genet
A.2009, 149A(7):1375-81.

Goula AV, Berquist BR, Wilson DM 3rd, Wheeler VC, Trottier Y, Merienne K (2009). Stoichiometry of base excision repair proteins correlates with increased somatic CAG instability in striatum over cerebellum In Huntington's disease transgenic mice. PLoS Genet. Dec;5(12):e1000749. Epub 2009 Dec 4.

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 (2010). A novel approach to investigate tissue-specific trinucleotide repeat instability. BMC Syst Biol. Mar 19;4(1):29.

Fossale E, Seong IS, Coser KR, Shioda T, Kohane IS, Wheeler VC, Gusella JF, Macdonald ME, Lee JM (2011). Differential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative. Hum Mol Genet. Aug 24. [Epub ahead of print]

Lee, JM, Pinto RM, Gillis T, St.Claire JC and Wheeler VC (2011). 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