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Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice.

Zheng S, Clabough EB, Sarkar S, Futter M, Rubinsztein DC, Zeitlin SO.

PLoS Genet. 2010 Feb 5;6(2):e1000838. doi: 10.1371/journal.pgen.1000838.


Expanded-polyglutamine huntingtin protein suppresses the secretion and production of a chemokine (CCL5/RANTES) by astrocytes.

Chou SY, Weng JY, Lai HL, Liao F, Sun SH, Tu PH, Dickson DW, Chern Y.

J Neurosci. 2008 Mar 26;28(13):3277-90. doi: 10.1523/JNEUROSCI.0116-08.2008.


Lack of huntingtin promotes neural stem cells differentiation into glial cells while neurons expressing huntingtin with expanded polyglutamine tracts undergo cell death.

Conforti P, Camnasio S, Mutti C, Valenza M, Thompson M, Fossale E, Zeitlin S, MacDonald ME, Zuccato C, Cattaneo E.

Neurobiol Dis. 2013 Feb;50:160-70. doi: 10.1016/j.nbd.2012.10.015. Epub 2012 Oct 23.


Mouse Huntington's disease gene homolog (Hdh).

Barnes GT, Duyao MP, Ambrose CM, McNeil S, Persichetti F, Srinidhi J, Gusella JF, MacDonald ME.

Somat Cell Mol Genet. 1994 Mar;20(2):87-97.


Elucidating a normal function of huntingtin by functional and microarray analysis of huntingtin-null mouse embryonic fibroblasts.

Zhang H, Das S, Li QZ, Dragatsis I, Repa J, Zeitlin S, Hajnóczky G, Bezprozvanny I.

BMC Neurosci. 2008 Apr 15;9:38. doi: 10.1186/1471-2202-9-38.


Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes.

Nasir J, Floresco SB, O'Kusky JR, Diewert VM, Richman JM, Zeisler J, Borowski A, Marth JD, Phillips AG, Hayden MR.

Cell. 1995 Jun 2;81(5):811-23.


The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin.

Auerbach W, Hurlbert MS, Hilditch-Maguire P, Wadghiri YZ, Wheeler VC, Cohen SI, Joyner AL, MacDonald ME, Turnbull DH.

Hum Mol Genet. 2001 Oct 15;10(22):2515-23.


Mouse Huntington's disease homolog mRNA levels: variation and allele effects.

Dixon KT, Cearley JA, Hunter JM, Detloff PJ.

Gene Expr. 2004;11(5-6):221-31.


Neocortical neurons cultured from mice with expanded CAG repeats in the huntingtin gene: unaltered vulnerability to excitotoxins and other insults.

Snider BJ, Moss JL, Revilla FJ, Lee CS, Wheeler VC, Macdonald ME, Choi DW.

Neuroscience. 2003;120(3):617-25.


Inactivation of the Huntington's disease gene (Hdh) impairs anterior streak formation and early patterning of the mouse embryo.

Woda JM, Calzonetti T, Hilditch-Maguire P, Duyao MP, Conlon RA, MacDonald ME.

BMC Dev Biol. 2005 Aug 18;5:17.


Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease.

Van Raamsdonk JM, Pearson J, Rogers DA, Bissada N, Vogl AW, Hayden MR, Leavitt BR.

Hum Mol Genet. 2005 May 15;14(10):1379-92. Epub 2005 Apr 13.


Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease.

Weiss A, Klein C, Woodman B, Sathasivam K, Bibel M, Régulier E, Bates GP, Paganetti P.

J Neurochem. 2008 Feb;104(3):846-58. Epub 2007 Nov 6.


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.


Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice.

Fossale E, Wheeler VC, Vrbanac V, Lebel LA, Teed A, Mysore JS, Gusella JF, MacDonald ME, Persichetti F.

Hum Mol Genet. 2002 Sep 15;11(19):2233-41.


Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice.

Neveklovska M, Clabough EB, Steffan JS, Zeitlin SO.

J Huntingtons Dis. 2012;1(1):71-87. doi: 10.3233/JHD-2012-120016.


Suppression of mutant Huntingtin aggregate formation by Cdk5/p35 through the effect on microtubule stability.

Kaminosono S, Saito T, Oyama F, Ohshima T, Asada A, Nagai Y, Nukina N, Hisanaga S.

J Neurosci. 2008 Aug 27;28(35):8747-55. doi: 10.1523/JNEUROSCI.0973-08.2008.

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