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Dysfunction of the ubiquitin ligase Ube3a may be associated with synaptic pathophysiology in a mouse model of Huntington disease.

Maheshwari M, Samanta A, Godavarthi SK, Mukherjee R, Jana NR.

J Biol Chem. 2012 Aug 24;287(35):29949-57. doi: 10.1074/jbc.M112.371724. Epub 2012 Jul 11.


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.


Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice.

Wang J, Wang CE, Orr A, Tydlacka S, Li SH, Li XJ.

J Cell Biol. 2008 Mar 24;180(6):1177-89. doi: 10.1083/jcb.200709080.


Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease.

Milnerwood AJ, Cummings DM, Dallérac GM, Brown JY, Vatsavayai SC, Hirst MC, Rezaie P, Murphy KP.

Hum Mol Genet. 2006 May 15;15(10):1690-703. Epub 2006 Apr 6.


Deficiency of Ube3a in Huntington's disease mice brain increases aggregate load and accelerates disease pathology.

Maheshwari M, Shekhar S, Singh BK, Jamal I, Vatsa N, Kumar V, Sharma A, Jana NR.

Hum Mol Genet. 2014 Dec 1;23(23):6235-45. doi: 10.1093/hmg/ddu343. Epub 2014 Jul 4.


Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation.

Murphy KP, Carter RJ, Lione LA, Mangiarini L, Mahal A, Bates GP, Dunnett SB, Morton AJ.

J Neurosci. 2000 Jul 1;20(13):5115-23.


Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats.

Menalled LB, Sison JD, Dragatsis I, Zeitlin S, Chesselet MF.

J Comp Neurol. 2003 Oct 6;465(1):11-26.


Striatal synaptic dysfunction and hippocampal plasticity deficits in the Hu97/18 mouse model of Huntington disease.

Kolodziejczyk K, Parsons MP, Southwell AL, Hayden MR, Raymond LA.

PLoS One. 2014 Apr 11;9(4):e94562. doi: 10.1371/journal.pone.0094562. eCollection 2014.


Dysregulation of synaptic proteins, dendritic spine abnormalities and pathological plasticity of synapses as experience-dependent mediators of cognitive and psychiatric symptoms in Huntington's disease.

Nithianantharajah J, Hannan AJ.

Neuroscience. 2013 Oct 22;251:66-74. doi: 10.1016/j.neuroscience.2012.05.043. Epub 2012 May 24. Review.


Cellular and subcellular localization of Huntingtin [corrected] aggregates in the brain of a rat transgenic for Huntington disease.

Petrasch-Parwez E, Nguyen HP, Löbbecke-Schumacher M, Habbes HW, Wieczorek S, Riess O, Andres KH, Dermietzel R, Von Hörsten S.

J Comp Neurol. 2007 Apr 10;501(5):716-30. Erratum in: J Comp Neurol. 2007 May 20;502(3):483.


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.


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.


Weight loss in Huntington disease increases with higher CAG repeat number.

Aziz NA, van der Burg JM, Landwehrmeyer GB, Brundin P, Stijnen T; EHDI Study Group, Roos RA.

Neurology. 2008 Nov 4;71(19):1506-13. doi: 10.1212/01.wnl.0000334276.09729.0e.


Differential changes in thalamic and cortical excitatory synapses onto striatal spiny projection neurons in a Huntington disease mouse model.

Kolodziejczyk K, Raymond LA.

Neurobiol Dis. 2016 Feb;86:62-74. doi: 10.1016/j.nbd.2015.11.020. Epub 2015 Nov 24.


Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease.

Zhang H, Li Q, Graham RK, Slow E, Hayden MR, Bezprozvanny I.

Neurobiol Dis. 2008 Jul;31(1):80-8. doi: 10.1016/j.nbd.2008.03.010. Epub 2008 Apr 16.


The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease.

Zeng L, Tallaksen-Greene SJ, Wang B, Albin RL, Paulson HL.

J Huntingtons Dis. 2013;2(2):201-15. doi: 10.3233/JHD-130058.


Polyglutamine-modulated striatal calpain activity in YAC transgenic huntington disease mouse model: impact on NMDA receptor function and toxicity.

Cowan CM, Fan MM, Fan J, Shehadeh J, Zhang LY, Graham RK, Hayden MR, Raymond LA.

J Neurosci. 2008 Nov 26;28(48):12725-35. doi: 10.1523/JNEUROSCI.4619-08.2008.


Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism.

Spires TL, Grote HE, Varshney NK, Cordery PM, van Dellen A, Blakemore C, Hannan AJ.

J Neurosci. 2004 Mar 3;24(9):2270-6.


Synaptic dysfunction in Huntington's disease: a new perspective.

Smith R, Brundin P, Li JY.

Cell Mol Life Sci. 2005 Sep;62(17):1901-12. Review.


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