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Progressive synaptic pathology of motor cortical neurons in a BAC transgenic mouse model of Huntington's disease.

Spampanato J, Gu X, Yang XW, Mody I.

Neuroscience. 2008 Dec 2;157(3):606-20. doi: 10.1016/j.neuroscience.2008.09.020. Epub 2008 Sep 18.


Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.

Gray M, Shirasaki DI, Cepeda C, André VM, Wilburn B, Lu XH, Tao J, Yamazaki I, Li SH, Sun YE, Li XJ, Levine MS, Yang XW.

J Neurosci. 2008 Jun 11;28(24):6182-95. doi: 10.1523/JNEUROSCI.0857-08.2008.


Synaptic scaling up in medium spiny neurons of aged BACHD mice: A slow-progression model of Huntington's disease.

Rocher AB, Gubellini P, Merienne N, Boussicault L, Petit F, Gipchtein P, Jan C, Hantraye P, Brouillet E, Bonvento G.

Neurobiol Dis. 2016 Feb;86:131-9. doi: 10.1016/j.nbd.2015.10.016. Epub 2015 Nov 25.


Cortical efferents lacking mutant huntingtin improve striatal neuronal activity and behavior in a conditional mouse model of Huntington's disease.

Estrada-Sánchez AM, Burroughs CL, Cavaliere S, Barton SJ, Chen S, Yang XW, Rebec GV.

J Neurosci. 2015 Mar 11;35(10):4440-51. doi: 10.1523/JNEUROSCI.2812-14.2015.


The mGluR5 positive allosteric modulator, CDPPB, ameliorates pathology and phenotypic signs of a mouse model of Huntington's disease.

Doria JG, de Souza JM, Andrade JN, Rodrigues HA, Guimaraes IM, Carvalho TG, Guatimosim C, Dobransky T, Ribeiro FM.

Neurobiol Dis. 2015 Jan;73:163-73. doi: 10.1016/j.nbd.2014.08.021. Epub 2014 Aug 24.


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.


Impaired development of cortico-striatal synaptic connectivity in a cell culture model of Huntington's disease.

Buren C, Parsons MP, Smith-Dijak A, Raymond LA.

Neurobiol Dis. 2016 Mar;87:80-90. doi: 10.1016/j.nbd.2015.12.009. Epub 2015 Dec 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.


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.


Sex Differences in Circadian Dysfunction in the BACHD Mouse Model of Huntington's Disease.

Kuljis DA, Gad L, Loh DH, MacDowell Kaswan Z, Hitchcock ON, Ghiani CA, Colwell CS.

PLoS One. 2016 Feb 12;11(2):e0147583. doi: 10.1371/journal.pone.0147583. eCollection 2016.


Mice lacking the transcriptional coactivator PGC-1α exhibit alterations in inhibitory synaptic transmission in the motor cortex.

Dougherty SE, Bartley AF, Lucas EK, Hablitz JJ, Dobrunz LE, Cowell RM.

Neuroscience. 2014 Jun 20;271:137-48. doi: 10.1016/j.neuroscience.2014.04.023. Epub 2014 Apr 24.


A novel BACHD transgenic rat exhibits characteristic neuropathological features of Huntington disease.

Yu-Taeger L, Petrasch-Parwez E, Osmand AP, Redensek A, Metzger S, Clemens LE, Park L, Howland D, Calaminus C, Gu X, Pichler B, Yang XW, Riess O, Nguyen HP.

J Neurosci. 2012 Oct 31;32(44):15426-38. doi: 10.1523/JNEUROSCI.1148-12.2012.


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.


Maternal loss of Ube3a produces an excitatory/inhibitory imbalance through neuron type-specific synaptic defects.

Wallace ML, Burette AC, Weinberg RJ, Philpot BD.

Neuron. 2012 Jun 7;74(5):793-800. doi: 10.1016/j.neuron.2012.03.036.


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.


Changes in structure and function of diaphragm neuromuscular junctions from BACHD mouse model for Huntington's disease.

de Aragão BC, Rodrigues HA, Valadão PA, Camargo W, Naves LA, Ribeiro FM, Guatimosim C.

Neurochem Int. 2016 Feb;93:64-72. doi: 10.1016/j.neuint.2015.12.013. Epub 2016 Jan 18.


Maintenance of basal levels of autophagy in Huntington's disease mouse models displaying metabolic dysfunction.

Baldo B, Soylu R, Petersén A.

PLoS One. 2013 Dec 20;8(12):e83050. doi: 10.1371/journal.pone.0083050. eCollection 2013.


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.


Non-fibrillar beta-amyloid abates spike-timing-dependent synaptic potentiation at excitatory synapses in layer 2/3 of the neocortex by targeting postsynaptic AMPA receptors.

Shemer I, Holmgren C, Min R, Fülöp L, Zilberter M, Sousa KM, Farkas T, Härtig W, Penke B, Burnashev N, Tanila H, Zilberter Y, Harkany T.

Eur J Neurosci. 2006 Apr;23(8):2035-47.


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