Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.
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Multiple Aspects of Gene Dysregulation in Huntington's DiseaseThe HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice.Current status of PET imaging in Huntington's diseaseMouse models of polyglutamine diseases: review and data table. Part IPossible involvement of self-defense mechanisms in the preferential vulnerability of the striatum in Huntington's diseaseComparative study of naturally occurring huntingtin fragments in Drosophila points to exon 1 as the most pathogenic species in Huntington's diseaseN17 Modifies mutant Huntingtin nuclear pathogenesis and severity of disease in HD BAC transgenic miceThe Wnt receptor Ryk reduces neuronal and cell survival capacity by repressing FOXO activity during the early phases of mutant huntingtin pathogenicityHDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegenerationEfficacy of selective PDE4D negative allosteric modulators in the object retrieval task in female cynomolgus monkeys (Macaca fascicularis)Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HDCorrelations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's diseaseDecreased striatal RGS2 expression is neuroprotective in Huntington's disease (HD) and exemplifies a compensatory aspect of HD-induced gene regulationSIRT2 ablation has no effect on tubulin acetylation in brain, cholesterol biosynthesis or the progression of Huntington's disease phenotypes in vivoGenome-wide loss of 5-hmC is a novel epigenetic feature of Huntington's diseaseGenetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's diseaseHdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington's diseaseExtensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease miceElucidating a normal function of huntingtin by functional and microarray analysis of huntingtin-null mouse embryonic fibroblasts.Expression analysis of novel striatal-enriched genes in Huntington disease.Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models.Preclinical assessment of CNS drug action using eye movements in mice.RGS4 is required for dopaminergic control of striatal LTD and susceptibility to parkinsonian motor deficitsBDNF-TrkB signaling in striatopallidal neurons controls inhibition of locomotor behavior.Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's diseaseTwo-transcript gene expression classifiers in the diagnosis and prognosis of human diseases.Cross-species and cross-platform gene expression studies with the Bioconductor-compliant R package 'annotationTools'Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington's disease.Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's diseaseFormation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease.HIF prolyl hydroxylase inhibitors prevent neuronal death induced by mitochondrial toxins: therapeutic implications for Huntington's disease and Alzheimer's disease.Decreased Lin7b expression in layer 5 pyramidal neurons may contribute to impaired corticostriatal connectivity in huntington disease.The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patientsNonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice.Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.Analysis of chaperone mRNA expression in the adult mouse brain by meta analysis of the Allen Brain Atlas.Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysisHuntington disease models and human neuropathology: similarities and differencesCalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease.Differential Alteration in Expression of Striatal GABAAR Subunits in Mouse Models of Huntington's Disease
P2860
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P2860
Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年学术文章
@wuu
2007年学术文章
@zh-cn
2007年学术文章
@zh-hans
2007年学术文章
@zh-my
2007年学术文章
@zh-sg
2007年學術文章
@yue
2007年學術文章
@zh
2007年學術文章
@zh-hant
name
Mutant huntingtin's effects on ...... r wild-type huntingtin dosage.
@en
Mutant huntingtin's effects on ...... r wild-type huntingtin dosage.
@nl
type
label
Mutant huntingtin's effects on ...... r wild-type huntingtin dosage.
@en
Mutant huntingtin's effects on ...... r wild-type huntingtin dosage.
@nl
prefLabel
Mutant huntingtin's effects on ...... r wild-type huntingtin dosage.
@en
Mutant huntingtin's effects on ...... r wild-type huntingtin dosage.
@nl
P2093
P2860
P50
P356
P1476
Mutant huntingtin's effects on ...... r wild-type huntingtin dosage.
@en
P2093
Alexandre Kuhn
Andrew D Strand
Darlene R Goldstein
James M Olson
Jang-Ho J Cha
Kirupa Sathasivam
Kristina Becanovic
Lesley Jones
Mauro Delorenzi
Peggy Shelbourne
P2860
P304
P356
10.1093/HMG/DDM133
P50
P577
2007-05-21T00:00:00Z