The PDE1/5 Inhibitor SCH-51866 Does Not Modify Disease Progression in the R6/2 Mouse Model of Huntington's DiseaseThe importance of integrating basic and clinical research toward the development of new therapies for Huntington diseaseA novel gene encoding an integral membrane protein is mutated in nephropathic cystinosisPolyglutamine expansion of huntingtin impairs its nuclear exportHuntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review seriesExendin-4 improves glycemic control, ameliorates brain and pancreatic pathologies, and extends survival in a mouse model of Huntington's diseaseHDAC4-myogenin axis as an important marker of HD-related skeletal muscle atrophySAHA decreases HDAC 2 and 4 levels in vivo and improves molecular phenotypes in the R6/2 mouse model of Huntington's diseaseHDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegenerationCorrelations 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 diseaseArfaptin 2 regulates the aggregation of mutant huntingtin proteinHuntingtin aggregation and toxicity in Huntington's diseaseThe Huntington's disease candidate region exhibits many different haplotypesFormation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutationSIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesisOral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivoElevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease miceHDAC4 does not act as a protein deacetylase in the postnatal murine brain in vivoGenetic 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 diseaseExon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic miceAggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brainIKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease.Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models.The telomeric 60 kb of chromosome arm 4p is homologous to telomeric regions on 13p, 15p, 21p, and 22p.Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction.Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease.Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease.Increased metabolism in the R6/2 mouse model of Huntington's disease.The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic 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 diseaseThe human homeobox gene HOX7 maps to chromosome 4p16.1 and may be implicated in Wolf-Hirschhorn syndrome.Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease.The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patientsProteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo.CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease.Contesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders.
P50
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P50
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British biologist
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British biologist
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British biologist
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Brits bioloog
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bióloga británica
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عالمة أحياء بريطانية
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Patricia Bates
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Gillian Patricia Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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Gillian Bates
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