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Items: 1 to 20 of 191

1.

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.

2.

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.

3.

Transgenic mice expressing mutated full-length HD cDNA: a paradigm for locomotor changes and selective neuronal loss in Huntington's disease.

Reddy PH, Charles V, Williams M, Miller G, Whetsell WO Jr, Tagle DA.

Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29;354(1386):1035-45.

4.

In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects.

Faideau M, Kim J, Cormier K, Gilmore R, Welch M, Auregan G, Dufour N, Guillermier M, Brouillet E, Hantraye P, Déglon N, Ferrante RJ, Bonvento G.

Hum Mol Genet. 2010 Aug 1;19(15):3053-67. doi: 10.1093/hmg/ddq212. Epub 2010 May 21.

5.

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.

6.

Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.

Kuhn A, Goldstein DR, Hodges A, Strand AD, Sengstag T, Kooperberg C, Becanovic K, Pouladi MA, Sathasivam K, Cha JH, Hannan AJ, Hayden MR, Leavitt BR, Dunnett SB, Ferrante RJ, Albin R, Shelbourne P, Delorenzi M, Augood SJ, Faull RL, Olson JM, Bates GP, Jones L, Luthi-Carter R.

Hum Mol Genet. 2007 Aug 1;16(15):1845-61. Epub 2007 May 21.

7.

HdhQ111 Mice Exhibit Tissue Specific Metabolite Profiles that Include Striatal Lipid Accumulation.

Carroll JB, Deik A, Fossale E, Weston RM, Guide JR, Arjomand J, Kwak S, Clish CB, MacDonald ME.

PLoS One. 2015 Aug 21;10(8):e0134465. doi: 10.1371/journal.pone.0134465. eCollection 2015.

8.

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.

9.

Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice.

Menalled LB, Sison JD, Wu Y, Olivieri M, Li XJ, Li H, Zeitlin S, Chesselet MF.

J Neurosci. 2002 Sep 15;22(18):8266-76.

10.

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.

11.

Transcription elongation and tissue-specific somatic CAG instability.

Goula AV, Stys A, Chan JP, Trottier Y, Festenstein R, Merienne K.

PLoS Genet. 2012;8(11):e1003051. doi: 10.1371/journal.pgen.1003051. Epub 2012 Nov 29.

12.

Glutathione redox cycle dysregulation in Huntington's disease knock-in striatal cells.

Ribeiro M, Rosenstock TR, Cunha-Oliveira T, Ferreira IL, Oliveira CR, Rego AC.

Free Radic Biol Med. 2012 Nov 15;53(10):1857-67. doi: 10.1016/j.freeradbiomed.2012.09.004. Epub 2012 Sep 14.

PMID:
22982598
13.

A critical role of astrocyte-mediated nuclear factor-κB-dependent inflammation in Huntington's disease.

Hsiao HY, Chen YC, Chen HM, Tu PH, Chern Y.

Hum Mol Genet. 2013 May 1;22(9):1826-42. doi: 10.1093/hmg/ddt036. Epub 2013 Jan 30.

14.

HD CAG-correlated gene expression changes support a simple dominant gain of function.

Jacobsen JC, Gregory GC, Woda JM, Thompson MN, Coser KR, Murthy V, Kohane IS, Gusella JF, Seong IS, MacDonald ME, Shioda T, Lee JM.

Hum Mol Genet. 2011 Jul 15;20(14):2846-60. doi: 10.1093/hmg/ddr195. Epub 2011 May 2.

15.

Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.

Yu ZX, Li SH, Evans J, Pillarisetti A, Li H, Li XJ.

J Neurosci. 2003 Mar 15;23(6):2193-202.

16.

Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease.

Ho LW, Brown R, Maxwell M, Wyttenbach A, Rubinsztein DC.

J Med Genet. 2001 Jul;38(7):450-2.

17.

Lack of huntingtin promotes neural stem cells differentiation into glial cells while neurons expressing huntingtin with expanded polyglutamine tracts undergo cell death.

Conforti P, Camnasio S, Mutti C, Valenza M, Thompson M, Fossale E, Zeitlin S, MacDonald ME, Zuccato C, Cattaneo E.

Neurobiol Dis. 2013 Feb;50:160-70. doi: 10.1016/j.nbd.2012.10.015. Epub 2012 Oct 23.

PMID:
23089356
18.

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.

19.

Novel BAC Mouse Model of Huntington's Disease with 225 CAG Repeats Exhibits an Early Widespread and Stable Degenerative Phenotype.

Wegrzynowicz M, Bichell TJ, Soares BD, Loth MK, McGlothan JS, Mori S, Alikhan FS, Hua K, Coughlin JM, Holt HK, Jetter CS, Pomper MG, Osmand AP, Guilarte TR, Bowman AB.

J Huntingtons Dis. 2015;4(1):17-36.

20.

Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin.

Carroll JB, Warby SC, Southwell AL, Doty CN, Greenlee S, Skotte N, Hung G, Bennett CF, Freier SM, Hayden MR.

Mol Ther. 2011 Dec;19(12):2178-85. doi: 10.1038/mt.2011.201. Epub 2011 Oct 4.

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