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Significantly differential diffusion of neuropathological aggregates in the brain of transgenic mice carrying N-terminal mutant huntingtin fused with green fluorescent protein.

Cheng PH, Li CL, Her LS, Chang YF, Chan AW, Chen CM, Yang SH.

Brain Struct Funct. 2013 Jan;218(1):283-94. doi: 10.1007/s00429-012-0401-x. Epub 2012 Mar 16.


Pathogenic cellular phenotypes are germline transmissible in a transgenic primate model of Huntington's disease.

Putkhao K, Kocerha J, Cho IK, Yang J, Parnpai R, Chan AW.

Stem Cells Dev. 2013 Apr 15;22(8):1198-205. doi: 10.1089/scd.2012.0469. Epub 2013 Jan 15.


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.


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.


Towards a transgenic model of Huntington's disease in a non-human primate.

Yang SH, Cheng PH, Banta H, Piotrowska-Nitsche K, Yang JJ, Cheng EC, Snyder B, Larkin K, Liu J, Orkin J, Fang ZH, Smith Y, Bachevalier J, Zola SM, Li SH, Li XJ, Chan AW.

Nature. 2008 Jun 12;453(7197):921-4. doi: 10.1038/nature06975. Epub 2008 May 18.


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.


Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin.

Tanaka Y, Igarashi S, Nakamura M, Gafni J, Torcassi C, Schilling G, Crippen D, Wood JD, Sawa A, Jenkins NA, Copeland NG, Borchelt DR, Ross CA, Ellerby LM.

Neurobiol Dis. 2006 Feb;21(2):381-91. Epub 2005 Sep 16.


Selective degeneration in YAC mouse models of Huntington disease.

Van Raamsdonk JM, Warby SC, Hayden MR.

Brain Res Bull. 2007 Apr 30;72(2-3):124-31. Epub 2006 Nov 16. Review.


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.


Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease.

Van Raamsdonk JM, Murphy Z, Slow EJ, Leavitt BR, Hayden MR.

Hum Mol Genet. 2005 Dec 15;14(24):3823-35. Epub 2005 Nov 8.


The Differential Profiling of Ubiquitin-Proteasome and Autophagy Systems in Different Tissues before the Onset of Huntington's Disease Models.

Her LS, Lin JY, Fu MH, Chang YF, Li CL, Tang TY, Jhang YL, Chang CY, Shih MC, Cheng PH, Yang SH.

Brain Pathol. 2015 Jul;25(4):481-90. doi: 10.1111/bpa.12191. Epub 2014 Nov 6.


Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease.

Hermel E, Gafni J, Propp SS, Leavitt BR, Wellington CL, Young JE, Hackam AS, Logvinova AV, Peel AL, Chen SF, Hook V, Singaraja R, Krajewski S, Goldsmith PC, Ellerby HM, Hayden MR, Bredesen DE, Ellerby LM.

Cell Death Differ. 2004 Apr;11(4):424-38.


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.


Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.

Sathasivam K, Neueder A, Gipson TA, Landles C, Benjamin AC, Bondulich MK, Smith DL, Faull RL, Roos RA, Howland D, Detloff PJ, Housman DE, Bates GP.

Proc Natl Acad Sci U S A. 2013 Feb 5;110(6):2366-70. doi: 10.1073/pnas.1221891110. Epub 2013 Jan 22.


Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length.

Hansson O, Castilho RF, Korhonen L, Lindholm D, Bates GP, Brundin P.

J Neurochem. 2001 Aug;78(4):694-703.


Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment.

Gafni J, Papanikolaou T, Degiacomo F, Holcomb J, Chen S, Menalled L, Kudwa A, Fitzpatrick J, Miller S, Ramboz S, Tuunanen PI, Lehtimäki KK, Yang XW, Park L, Kwak S, Howland D, Park H, Ellerby LM.

J Neurosci. 2012 May 30;32(22):7454-65. doi: 10.1523/JNEUROSCI.6379-11.2012.


Increased caspase-2, calpain activations and decreased mitochondrial complex II activity in cells expressing exogenous huntingtin exon 1 containing CAG repeat in the pathogenic range.

Majumder P, Raychaudhuri S, Chattopadhyay B, Bhattacharyya NP.

Cell Mol Neurobiol. 2007 Dec;27(8):1127-45. Epub 2007 Sep 28.


Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice.

Bradford J, Shin JY, Roberts M, Wang CE, Sheng G, Li S, Li XJ.

J Biol Chem. 2010 Apr 2;285(14):10653-61. doi: 10.1074/jbc.M109.083287. Epub 2010 Feb 9.

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