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Dysfunction of the CNS-heart axis in mouse models of Huntington's disease.

Mielcarek M, Inuabasi L, Bondulich MK, Muller T, Osborne GF, Franklin SA, Smith DL, Neueder A, Rosinski J, Rattray I, Protti A, Bates GP.

PLoS Genet. 2014 Aug 7;10(8):e1004550. doi: 10.1371/journal.pgen.1004550. eCollection 2014 Aug.


HDAC4-myogenin axis as an important marker of HD-related skeletal muscle atrophy.

Mielcarek M, Toczek M, Smeets CJ, Franklin SA, Bondulich MK, Jolinon N, Muller T, Ahmed M, Dick JR, Piotrowska I, Greensmith L, Smolenski RT, Bates GP.

PLoS Genet. 2015 Mar 6;11(3):e1005021. doi: 10.1371/journal.pgen.1005021. eCollection 2015 Mar.


Huntington's disease induced cardiac amyloidosis is reversed by modulating protein folding and oxidative stress pathways in the Drosophila heart.

Melkani GC, Trujillo AS, Ramos R, Bodmer R, Bernstein SI, Ocorr K.

PLoS Genet. 2013;9(12):e1004024. doi: 10.1371/journal.pgen.1004024. Epub 2013 Dec 19.


The Huntington's disease-related cardiomyopathy prevents a hypertrophic response in the R6/2 mouse model.

Mielcarek M, Bondulich MK, Inuabasi L, Franklin SA, Muller T, Bates GP.

PLoS One. 2014 Sep 30;9(9):e108961. doi: 10.1371/journal.pone.0108961. eCollection 2014.


Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease.

Moffitt H, McPhail GD, Woodman B, Hobbs C, Bates GP.

PLoS One. 2009 Nov 30;4(11):e8025. doi: 10.1371/journal.pone.0008025.


Cardiac dysfunction in the R6/2 mouse model of Huntington's disease.

Mihm MJ, Amann DM, Schanbacher BL, Altschuld RA, Bauer JA, Hoyt KR.

Neurobiol Dis. 2007 Feb;25(2):297-308. Epub 2006 Nov 27.


Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease.

Wang CE, Tydlacka S, Orr AL, Yang SH, Graham RK, Hayden MR, Li S, Chan AW, Li XJ.

Hum Mol Genet. 2008 Sep 1;17(17):2738-51. doi: 10.1093/hmg/ddn175. Epub 2008 Jun 16.


Antisense oligonucleotide-mediated correction of transcriptional dysregulation is correlated with behavioral benefits in the YAC128 mouse model of Huntington's disease.

Stanek LM, Yang W, Angus S, Sardi PS, Hayden MR, Hung GH, Bennett CF, Cheng SH, Shihabuddin LS.

J Huntingtons Dis. 2013;2(2):217-28. doi: 10.3233/JHD-130057.


Transcriptional dysregulation of coding and non-coding genes in cellular models of Huntington's disease.

Bithell A, Johnson R, Buckley NJ.

Biochem Soc Trans. 2009 Dec;37(Pt 6):1270-5. doi: 10.1042/BST0371270. Review.


In vivo cell-autonomous transcriptional abnormalities revealed in mice expressing mutant huntingtin in striatal but not cortical neurons.

Thomas EA, Coppola G, Tang B, Kuhn A, Kim S, Geschwind DH, Brown TB, Luthi-Carter R, Ehrlich ME.

Hum Mol Genet. 2011 Mar 15;20(6):1049-60. doi: 10.1093/hmg/ddq548. Epub 2010 Dec 20.


Direct evidence of progressive cardiac dysfunction in a transgenic mouse model of Huntington's disease.

Wood NI, Sawiak SJ, Buonincontri G, Niu Y, Kane AD, Carpenter TA, Giussani DA, Morton AJ.

J Huntingtons Dis. 2012 Jan;1(1). doi: 10.3233/JHD-2012-120004.


Mutant huntingtin gene-dose impacts on aggregate deposition, DARPP32 expression and neuroinflammation in HdhQ150 mice.

Young D, Mayer F, Vidotto N, Schweizer T, Berth R, Abramowski D, Shimshek DR, van der Putten PH, Schmid P.

PLoS One. 2013 Sep 23;8(9):e75108. doi: 10.1371/journal.pone.0075108. eCollection 2013.


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.


Ubiquilin-1 overexpression increases the lifespan and delays accumulation of Huntingtin aggregates in the R6/2 mouse model of Huntington's disease.

Safren N, El Ayadi A, Chang L, Terrillion CE, Gould TD, Boehning DF, Monteiro MJ.

PLoS One. 2014 Jan 27;9(1):e87513. doi: 10.1371/journal.pone.0087513. eCollection 2014.


Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models.

Phan J, Hickey MA, Zhang P, Chesselet MF, Reue K.

Hum Mol Genet. 2009 Mar 15;18(6):1006-16. doi: 10.1093/hmg/ddn428. Epub 2009 Jan 5.


Huntington's disease: the current state of research with peripheral tissues.

Sassone J, Colciago C, Cislaghi G, Silani V, Ciammola A.

Exp Neurol. 2009 Oct;219(2):385-97. doi: 10.1016/j.expneurol.2009.05.012. Epub 2009 May 19. Review.


Expression of expanded CAG transcripts triggers nucleolar stress in Huntington's disease.

Tsoi H, Chan HY.

Cerebellum. 2013 Jun;12(3):310-2. doi: 10.1007/s12311-012-0447-6.


Selective neuronal degeneration in Huntington's disease.

Cowan CM, Raymond LA.

Curr Top Dev Biol. 2006;75:25-71. Review.

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