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Items: 48

1.

Transcriptional regulatory networks underlying gene expression changes in Huntington's disease.

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.

Mol Syst Biol. 2018 Mar 26;14(3):e7435. doi: 10.15252/msb.20167435.

2.

HttQ111/+ Huntington's Disease Knock-in Mice Exhibit Brain Region-Specific Morphological Changes and Synaptic Dysfunction.

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.

J Huntingtons Dis. 2018;7(1):17-33. doi: 10.3233/JHD-170282.

3.

A modifier of Huntington's disease onset at the MLH1 locus.

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.

Hum Mol Genet. 2017 Oct 1;26(19):3859-3867. doi: 10.1093/hmg/ddx286.

PMID:
28934397
4.

Haplotype-based stratification of Huntington's disease.

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.

Eur J Hum Genet. 2017 Nov;25(11):1202-1209. doi: 10.1038/ejhg.2017.125. Epub 2017 Aug 23.

PMID:
28832564
5.

Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport.

Gasset-Rosa F, Chillon-Marinas C, Goginashvili A, Atwal RS, Artates JW, Tabet R, Wheeler VC, Bang AG, Cleveland DW, Lagier-Tourenne C.

Neuron. 2017 Apr 5;94(1):48-57.e4. doi: 10.1016/j.neuron.2017.03.027.

6.

Corrigendum: Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington's disease.

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.

Sci Rep. 2017 Mar 28;7:44960. doi: 10.1038/srep44960. No abstract available.

7.

High resolution time-course mapping of early transcriptomic, molecular and cellular phenotypes in Huntington's disease CAG knock-in mice across multiple genetic backgrounds.

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.

Hum Mol Genet. 2017 Mar 1;26(5):913-922. doi: 10.1093/hmg/ddx006.

PMID:
28334820
8.

Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington's disease.

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.

Sci Rep. 2017 Feb 8;7:41570. doi: 10.1038/srep41570.

9.

Genetic Contributors to Intergenerational CAG Repeat Instability in Huntington's Disease Knock-In Mice.

Neto JL, Lee JM, Afridi A, Gillis T, Guide JR, Dempsey S, Lager B, Alonso I, Wheeler VC, Pinto RM.

Genetics. 2017 Feb;205(2):503-516. doi: 10.1534/genetics.116.195578. Epub 2016 Dec 2.

10.

Large-scale phenome analysis defines a behavioral signature for Huntington's disease genotype in mice.

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.

Nat Biotechnol. 2016 Aug;34(8):838-44. doi: 10.1038/nbt.3587. Epub 2016 Jul 4.

PMID:
27376585
11.

The Genetic Modifiers of Motor OnsetAge (GeM MOA) Website: Genome-wide Association Analysis for Genetic Modifiers of Huntington's Disease.

Correia K, Harold D, Kim KH, Holmans P, Jones L, Orth M, Myers RH, Kwak S, Wheeler VC, MacDonald ME, Gusella JF, Lee JM.

J Huntingtons Dis. 2015;4(3):279-84. doi: 10.3233/JHD-150169.

12.

Chromosome substitution strain assessment of a Huntington's disease modifier locus.

Ramos EM, Kovalenko M, Guide JR, St Claire J, Gillis T, Mysore JS, Sequeiros J, Wheeler VC, Alonso I, MacDonald ME.

Mamm Genome. 2015 Apr;26(3-4):119-30. doi: 10.1007/s00335-014-9552-9. Epub 2015 Feb 3.

13.

Transcriptional consequences of 16p11.2 deletion and duplication in mouse cortex and multiplex autism families.

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.

Am J Hum Genet. 2014 Jun 5;94(6):870-83. doi: 10.1016/j.ajhg.2014.05.004.

14.

HD CAGnome: a search tool for huntingtin CAG repeat length-correlated genes.

Galkina EI, Shin A, Coser KR, Shioda T, Kohane IS, Seong IS, Wheeler VC, Gusella JF, Macdonald ME, Lee JM.

PLoS One. 2014 Apr 21;9(4):e95556. doi: 10.1371/journal.pone.0095556. eCollection 2014.

15.

A broad phenotypic screen identifies novel phenotypes driven by a single mutant allele in Huntington's disease CAG knock-in mice.

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.

PLoS One. 2013 Nov 22;8(11):e80923. doi: 10.1371/journal.pone.0080923. eCollection 2013.

16.

Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches.

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.

PLoS Genet. 2013 Oct;9(10):e1003930. doi: 10.1371/journal.pgen.1003930. Epub 2013 Oct 31.

17.

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.

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.

Hum Mol Genet. 2013 Aug 15;22(16):3227-38. doi: 10.1093/hmg/ddt176. Epub 2013 Apr 16.

18.

Msh2 acts in medium-spiny striatal neurons as an enhancer of CAG instability and mutant huntingtin phenotypes in Huntington's disease knock-in mice.

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.

PLoS One. 2012;7(9):e44273. doi: 10.1371/journal.pone.0044273. Epub 2012 Sep 7.

19.

Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous system.

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.

PLoS One. 2012;7(6):e38310. doi: 10.1371/journal.pone.0038310. Epub 2012 Jun 6.

20.

Early alterations of brain cellular energy homeostasis in Huntington disease models.

Mochel F, Durant B, Meng X, O'Callaghan J, Yu H, Brouillet E, Wheeler VC, Humbert S, Schiffmann R, Durr A.

J Biol Chem. 2012 Jan 6;287(2):1361-70. doi: 10.1074/jbc.M111.309849. Epub 2011 Nov 28.

21.

Quantification of age-dependent somatic CAG repeat instability in Hdh CAG knock-in mice reveals different expansion dynamics in striatum and liver.

Lee JM, Pinto RM, Gillis T, St Claire JC, Wheeler VC.

PLoS One. 2011;6(8):e23647. doi: 10.1371/journal.pone.0023647. Epub 2011 Aug 29.

22.

Differential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative.

Fossale E, Seong IS, Coser KR, Shioda T, Kohane IS, Wheeler VC, Gusella JF, MacDonald ME, Lee JM.

Hum Mol Genet. 2011 Nov 1;20(21):4258-67. doi: 10.1093/hmg/ddr355. Epub 2011 Aug 12.

23.

A novel approach to investigate tissue-specific trinucleotide repeat instability.

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.

BMC Syst Biol. 2010 Mar 19;4:29. doi: 10.1186/1752-0509-4-29.

24.

Stoichiometry of base excision repair proteins correlates with increased somatic CAG instability in striatum over cerebellum in Huntington's disease transgenic mice.

Goula AV, Berquist BR, Wilson DM 3rd, Wheeler VC, Trottier Y, Merienne K.

PLoS Genet. 2009 Dec;5(12):e1000749. doi: 10.1371/journal.pgen.1000749. Epub 2009 Dec 4.

25.

Huntingtin facilitates polycomb repressive complex 2.

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.

Hum Mol Genet. 2010 Feb 15;19(4):573-83. doi: 10.1093/hmg/ddp524. Epub 2009 Nov 23.

26.

Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset.

Swami M, Hendricks AE, Gillis T, Massood T, Mysore J, Myers RH, Wheeler VC.

Hum Mol Genet. 2009 Aug 15;18(16):3039-47. doi: 10.1093/hmg/ddp242. Epub 2009 May 23.

27.

Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes.

Dragileva E, Hendricks A, Teed A, Gillis T, Lopez ET, Friedberg EC, Kucherlapati R, Edelmann W, Lunetta KL, MacDonald ME, Wheeler VC.

Neurobiol Dis. 2009 Jan;33(1):37-47. doi: 10.1016/j.nbd.2008.09.014. Epub 2008 Sep 30.

28.

Factors associated with HD CAG repeat instability in Huntington disease.

Wheeler VC, Persichetti F, McNeil SM, Mysore JS, Mysore SS, MacDonald ME, Myers RH, Gusella JF, Wexler NS; US-Venezuela Collaborative Research Group.

J Med Genet. 2007 Nov;44(11):695-701. Epub 2007 Jul 27.

29.

Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study.

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.

BMC Med Genet. 2006 Aug 17;7:71.

30.

Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice.

Guidetti P, Bates GP, Graham RK, Hayden MR, Leavitt BR, MacDonald ME, Slow EJ, Wheeler VC, Woodman B, Schwarcz R.

Neurobiol Dis. 2006 Jul;23(1):190-7. Epub 2006 May 12.

PMID:
16697652
31.

Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice.

Lloret A, Dragileva E, Teed A, Espinola J, Fossale E, Gillis T, Lopez E, Myers RH, MacDonald ME, Wheeler VC.

Hum Mol Genet. 2006 Jun 15;15(12):2015-24. Epub 2006 May 10.

PMID:
16687439
32.

Huntington's disease.

MacDonald ME, Gines S, Gusella JF, Wheeler VC.

Neuromolecular Med. 2003;4(1-2):7-20. Review.

PMID:
14528049
33.

A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study.

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.

Am J Hum Genet. 2003 Sep;73(3):682-7. Epub 2003 Aug 1.

34.

Neocortical neurons cultured from mice with expanded CAG repeats in the huntingtin gene: unaltered vulnerability to excitotoxins and other insults.

Snider BJ, Moss JL, Revilla FJ, Lee CS, Wheeler VC, Macdonald ME, Choi DW.

Neuroscience. 2003;120(3):617-25.

PMID:
12895502
35.

Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice.

Gines S, Seong IS, Fossale E, Ivanova E, Trettel F, Gusella JF, Wheeler VC, Persichetti F, MacDonald ME.

Hum Mol Genet. 2003 Mar 1;12(5):497-508.

PMID:
12588797
36.

Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum.

Wheeler VC, Lebel LA, Vrbanac V, Teed A, te Riele H, MacDonald ME.

Hum Mol Genet. 2003 Feb 1;12(3):273-81.

PMID:
12554681
37.

The HD mutation does not alter neuronal death in the striatum of Hdh(Q92) knock-in mice after mild focal ischemia.

Namura S, Hirt L, Wheeler VC, McGinnis KM, Hilditch-Maguire P, Moskowitz MA, MacDonald ME, Persichetti F.

Neurobiol Dis. 2002 Oct;11(1):147-54.

PMID:
12460554
38.

Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice.

Fossale E, Wheeler VC, Vrbanac V, Lebel LA, Teed A, Mysore JS, Gusella JF, MacDonald ME, Persichetti F.

Hum Mol Genet. 2002 Sep 15;11(19):2233-41.

PMID:
12217951
39.

Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice.

Wheeler VC, Gutekunst CA, Vrbanac V, Lebel LA, Schilling G, Hersch S, Friedlander RM, Gusella JF, Vonsattel JP, Borchelt DR, MacDonald ME.

Hum Mol Genet. 2002 Mar 15;11(6):633-40.

PMID:
11912178
40.

The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin.

Auerbach W, Hurlbert MS, Hilditch-Maguire P, Wadghiri YZ, Wheeler VC, Cohen SI, Joyner AL, MacDonald ME, Turnbull DH.

Hum Mol Genet. 2001 Oct 15;10(22):2515-23.

PMID:
11709539
41.

Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells.

Trettel F, Rigamonti D, Hilditch-Maguire P, Wheeler VC, Sharp AH, Persichetti F, Cattaneo E, MacDonald ME.

Hum Mol Genet. 2000 Nov 22;9(19):2799-809.

PMID:
11092756
42.

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice.

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.

Hum Mol Genet. 2000 Mar 1;9(4):503-13.

PMID:
10699173
43.

Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse.

Wheeler VC, Auerbach W, White JK, Srinidhi J, Auerbach A, Ryan A, Duyao MP, Vrbanac V, Weaver M, Gusella JF, Joyner AL, MacDonald ME.

Hum Mol Genet. 1999 Jan;8(1):115-22.

PMID:
9887339
44.

Modification of the mouse mitochondrial genome by insertion of an exogenous gene.

Wheeler VC, Aitken M, Coutelle C.

Gene. 1997 Oct 1;198(1-2):203-9.

PMID:
9370282
45.
47.

Nondegradative in vitro labeling of plasmid DNA.

Wheeler VC, Coutelle C.

Anal Biochem. 1995 Mar 1;225(2):374-6. No abstract available.

PMID:
7762810
48.

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