Literature DB >> 10814708

Decreased expression of striatal signaling genes in a mouse model of Huntington's disease.

R Luthi-Carter1, A Strand, N L Peters, S M Solano, Z R Hollingsworth, A S Menon, A S Frey, B S Spektor, E B Penney, G Schilling, C A Ross, D R Borchelt, S J Tapscott, A B Young, J H Cha, J M Olson.   

Abstract

To understand gene expression changes mediated by a polyglutamine repeat expansion in the human huntingtin protein, we used oligonucleotide DNA arrays to profile approximately 6000 striatal mRNAs in the R6/2 mouse, a transgenic Huntington's disease (HD) model. We found diminished levels of mRNAs encoding components of the neurotransmitter, calcium and retinoid signaling pathways at both early and late symptomatic time points (6 and 12 weeks of age). We observed similar changes in gene expression in another HD mouse model (N171-82Q). These results demonstrate that mutant huntingtin directly or indirectly reduces the expression of a distinct set of genes involved in signaling pathways known to be critical to striatal neuron function.

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Year:  2000        PMID: 10814708     DOI: 10.1093/hmg/9.9.1259

Source DB:  PubMed          Journal:  Hum Mol Genet        ISSN: 0964-6906            Impact factor:   6.150


  193 in total

1.  The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription.

Authors:  J S Steffan; A Kazantsev; O Spasic-Boskovic; M Greenwald; Y Z Zhu; H Gohler; E E Wanker; G P Bates; D E Housman; L M Thompson
Journal:  Proc Natl Acad Sci U S A       Date:  2000-06-06       Impact factor: 11.205

2.  Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation.

Authors:  S Waelter; A Boeddrich; R Lurz; E Scherzinger; G Lueder; H Lehrach; E E Wanker
Journal:  Mol Biol Cell       Date:  2001-05       Impact factor: 4.138

3.  Striatal-enriched protein tyrosine phosphatase expression and activity in Huntington's disease: a STEP in the resistance to excitotoxicity.

Authors:  Ana Saavedra; Albert Giralt; Laura Rué; Xavier Xifró; Jian Xu; Zaira Ortega; José J Lucas; Paul J Lombroso; Jordi Alberch; Esther Pérez-Navarro
Journal:  J Neurosci       Date:  2011-06-01       Impact factor: 6.167

4.  Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative.

Authors:  Andrew T N Tebbenkamp; Cameron Green; Guilian Xu; Eileen M Denovan-Wright; Aaron C Rising; Susan E Fromholt; Hilda H Brown; Debbie Swing; Ronald J Mandel; Lino Tessarollo; David R Borchelt
Journal:  Hum Mol Genet       Date:  2011-04-22       Impact factor: 6.150

Review 5.  Huntingtin in health and disease.

Authors:  Anne B Young
Journal:  J Clin Invest       Date:  2003-02       Impact factor: 14.808

6.  Quantitative assessment of transcriptome differences between brain territories.

Authors:  Michel de Chaldée; Marie-Claude Gaillard; Nicolas Bizat; Jean-Marie Buhler; Olivier Manzoni; Joël Bockaert; Philippe Hantraye; Emmanuel Brouillet; Jean-Marc Elalouf
Journal:  Genome Res       Date:  2003-07       Impact factor: 9.043

Review 7.  Huntington's disease: a decade beyond gene discovery.

Authors:  Penelope Hogarth
Journal:  Curr Neurol Neurosci Rep       Date:  2003-07       Impact factor: 5.081

Review 8.  Functional aspects of cellular microcompartmentation in the development of neurodegeneration: mutation induced aberrant protein-protein associations.

Authors:  Judit Ovádi; Ferenc Orosz; Susan Hollán
Journal:  Mol Cell Biochem       Date:  2004 Jan-Feb       Impact factor: 3.396

9.  Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease.

Authors:  Tyisha Hathorn; Abigail Snyder-Keller; Anne Messer
Journal:  Neurobiol Dis       Date:  2010-08-22       Impact factor: 5.996

10.  Tracking brain palmitoylation change: predominance of glial change in a mouse model of Huntington's disease.

Authors:  Junmei Wan; Jeffrey N Savas; Amy F Roth; Shaun S Sanders; Roshni R Singaraja; Michael R Hayden; John R Yates; Nicholas G Davis
Journal:  Chem Biol       Date:  2013-11-07
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