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Items: 1 to 20 of 101

1.

Comparative analyses of Purkinje cell gene expression profiles reveal shared molecular abnormalities in models of different polyglutamine diseases.

Friedrich B, Euler P, Ziegler R, Kuhn A, Landwehrmeyer BG, Luthi-Carter R, Weiller C, Hellwig S, Zucker B.

Brain Res. 2012 Oct 24;1481:37-48. doi: 10.1016/j.brainres.2012.08.005. Epub 2012 Aug 17.

PMID:
22917585
2.

Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model.

Helmlinger D, Abou-Sleymane G, Yvert G, Rousseau S, Weber C, Trottier Y, Mandel JL, Devys D.

J Neurosci. 2004 Feb 25;24(8):1881-7.

3.

Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous bergmann glia degeneration.

Furrer SA, Mohanachandran MS, Waldherr SM, Chang C, Damian VA, Sopher BL, Garden GA, La Spada AR.

J Neurosci. 2011 Nov 9;31(45):16269-78. doi: 10.1523/JNEUROSCI.4000-11.2011.

4.

Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7.

Furrer SA, Waldherr SM, Mohanachandran MS, Baughn TD, Nguyen KT, Sopher BL, Damian VA, Garden GA, La Spada AR.

Hum Mol Genet. 2013 Mar 1;22(5):890-903. doi: 10.1093/hmg/dds495. Epub 2012 Nov 29.

5.

Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum.

Ebner BA, Ingram MA, Barnes JA, Duvick LA, Frisch JL, Clark HB, Zoghbi HY, Ebner TJ, Orr HT.

J Neurosci. 2013 Mar 27;33(13):5806-20. doi: 10.1523/JNEUROSCI.6311-11.2013.

6.

Gene expression analysis on a single cell level in Purkinje cells of Huntington's disease transgenic mice.

Euler P, Friedrich B, Ziegler R, Kuhn A, Lindenberg KS, Weiller C, Zucker B.

Neurosci Lett. 2012 May 23;517(1):7-12.

PMID:
22712074
7.

Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.

Ingram M, Wozniak EA, Duvick L, Yang R, Bergmann P, Carson R, O'Callaghan B, Zoghbi HY, Henzler C, Orr HT.

Neuron. 2016 Mar 16;89(6):1194-207. doi: 10.1016/j.neuron.2016.02.011. Epub 2016 Mar 3.

8.
9.

Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration.

Garden GA, La Spada AR.

Cerebellum. 2008;7(2):138-49. doi: 10.1007/s12311-008-0027-y. Review.

10.

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

Garden GA, Libby RT, Fu YH, Kinoshita Y, Huang J, Possin DE, Smith AC, Martinez RA, Fine GC, Grote SK, Ware CB, Einum DD, Morrison RS, Ptacek LJ, Sopher BL, La Spada AR.

J Neurosci. 2002 Jun 15;22(12):4897-905.

11.

Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases.

Branco J, Al-Ramahi I, Ukani L, Pérez AM, Fernandez-Funez P, Rincón-Limas D, Botas J.

Hum Mol Genet. 2008 Feb 1;17(3):376-90. Epub 2007 Nov 5.

PMID:
17984172
12.

Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease.

Chan EY, Luthi-Carter R, Strand A, Solano SM, Hanson SA, DeJohn MM, Kooperberg C, Chase KO, DiFiglia M, Young AB, Leavitt BR, Cha JH, Aronin N, Hayden MR, Olson JM.

Hum Mol Genet. 2002 Aug 15;11(17):1939-51.

PMID:
12165556
13.

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects.

Luthi-Carter R, Strand AD, Hanson SA, Kooperberg C, Schilling G, La Spada AR, Merry DE, Young AB, Ross CA, Borchelt DR, Olson JM.

Hum Mol Genet. 2002 Aug 15;11(17):1927-37.

PMID:
12165555
14.
15.

Disruption of Purkinje cell function prior to huntingtin accumulation and cell loss in an animal model of Huntington disease.

Dougherty SE, Reeves JL, Lucas EK, Gamble KL, Lesort M, Cowell RM.

Exp Neurol. 2012 Jul;236(1):171-8. doi: 10.1016/j.expneurol.2012.04.015. Epub 2012 May 2.

16.

Decreased expression of hypothalamic neuropeptides in Huntington disease transgenic mice with expanded polyglutamine-EGFP fluorescent aggregates.

Kotliarova S, Jana NR, Sakamoto N, Kurosawa M, Miyazaki H, Nekooki M, Doi H, Machida Y, Wong HK, Suzuki T, Uchikawa C, Kotliarov Y, Uchida K, Nagao Y, Nagaoka U, Tamaoka A, Oyanagi K, Oyama F, Nukina N.

J Neurochem. 2005 May;93(3):641-53.

17.

SUMO-1 interacts with mutant ataxin-1 and colocalizes to its aggregates in Purkinje cells of SCA1 transgenic mice.

Kang S, Hong S.

Arch Ital Biol. 2010 Dec;148(4):351-63. doi: 10.4449/aib.v148i4.1201.

PMID:
21308649
18.

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.

19.

Progressive retinal degeneration and dysfunction in R6 Huntington's disease mice.

Helmlinger D, Yvert G, Picaud S, Merienne K, Sahel J, Mandel JL, Devys D.

Hum Mol Genet. 2002 Dec 15;11(26):3351-9.

PMID:
12471061
20.

Relationship between ataxin-1 nuclear inclusions and Purkinje cell specific proteins in SCA-1 transgenic mice.

Vig PJ, Subramony SH, Qin Z, McDaniel DO, Fratkin JD.

J Neurol Sci. 2000 Mar 15;174(2):100-10.

PMID:
10727695

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