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In vivo identification of therapeutic constructs from pooled candidates in HD model mice.

Crook ZR, Housman DE.

J Huntingtons Dis. 2013;2(4):437-41. doi: 10.3233/JHD-130073.


AAV vector-mediated RNAi of mutant huntingtin expression is neuroprotective in a novel genetic rat model of Huntington's disease.

Franich NR, Fitzsimons HL, Fong DM, Klugmann M, During MJ, Young D.

Mol Ther. 2008 May;16(5):947-56. doi: 10.1038/mt.2008.50. Epub 2008 Mar 25.


Striatal expression of a calmodulin fragment improved motor function, weight loss, and neuropathology in the R6/2 mouse model of Huntington's disease.

Dai Y, Dudek NL, Li Q, Fowler SC, Muma NA.

J Neurosci. 2009 Sep 16;29(37):11550-9. doi: 10.1523/JNEUROSCI.3307-09.2009.


Dysregulation of dopamine receptor D2 as a sensitive measure for Huntington disease pathology in model mice.

Crook ZR, Housman DE.

Proc Natl Acad Sci U S A. 2012 May 8;109(19):7487-92. doi: 10.1073/pnas.1204542109. Epub 2012 Apr 23.


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.


Striatal pre-enkephalin overexpression improves Huntington's disease symptoms in the R6/2 mouse model of Huntington's disease.

Bissonnette S, Vaillancourt M, Hébert SS, Drolet G, Samadi P.

PLoS One. 2013 Sep 11;8(9):e75099. doi: 10.1371/journal.pone.0075099. eCollection 2013.


Dendritic spine instability leads to progressive neocortical spine loss in a mouse model of Huntington's disease.

Murmu RP, Li W, Holtmaat A, Li JY.

J Neurosci. 2013 Aug 7;33(32):12997-3009. doi: 10.1523/JNEUROSCI.5284-12.2013.


miR-196a ameliorates phenotypes of Huntington disease in cell, transgenic mouse, and induced pluripotent stem cell models.

Cheng PH, Li CL, Chang YF, Tsai SJ, Lai YY, Chan AW, Chen CM, Yang SH.

Am J Hum Genet. 2013 Aug 8;93(2):306-12. doi: 10.1016/j.ajhg.2013.05.025. Epub 2013 Jun 27.


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.


Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis.

Lee CY, Cantle JP, Yang XW.

FEBS J. 2013 Sep;280(18):4382-94. doi: 10.1111/febs.12418. Epub 2013 Jul 31. Review.


Differential morphology and composition of inclusions in the R6/2 mouse and PC12 cell models of Huntington's disease.

Wanderer J, Morton AJ.

Histochem Cell Biol. 2007 May;127(5):473-84. Epub 2007 Feb 7.


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.


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.


Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain.

Ginés S, Bosch M, Marco S, Gavaldà N, Díaz-Hernández M, Lucas JJ, Canals JM, Alberch J.

Eur J Neurosci. 2006 Feb;23(3):649-58.


Altered serotonin receptor expression is associated with depression-related behavior in the R6/1 transgenic mouse model of Huntington's disease.

Pang TY, Du X, Zajac MS, Howard ML, Hannan AJ.

Hum Mol Genet. 2009 Feb 15;18(4):753-66. doi: 10.1093/hmg/ddn385. Epub 2008 Nov 13.


Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs.

Yang D, Wang CE, Zhao B, Li W, Ouyang Z, Liu Z, Yang H, Fan P, O'Neill A, Gu W, Yi H, Li S, Lai L, Li XJ.

Hum Mol Genet. 2010 Oct 15;19(20):3983-94. doi: 10.1093/hmg/ddq313. Epub 2010 Jul 21.


Gene expression and behaviour in mouse models of HD.

Bowles KR, Brooks SP, Dunnett SB, Jones L.

Brain Res Bull. 2012 Jun 1;88(2-3):276-84. doi: 10.1016/j.brainresbull.2011.07.021. Epub 2011 Aug 10. Review.


Examination of mesenchymal stem cell-mediated RNAi transfer to Huntington's disease affected neuronal cells for reduction of huntingtin.

Olson SD, Kambal A, Pollock K, Mitchell GM, Stewart H, Kalomoiris S, Cary W, Nacey C, Pepper K, Nolta JA.

Mol Cell Neurosci. 2012 Mar;49(3):271-81. doi: 10.1016/j.mcn.2011.12.001. Epub 2011 Dec 8.


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.

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