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Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.
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J Neurosci
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2008
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Huntingtin-protein interactions and the pathogenesis of Huntington's disease.
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Trends Genet
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Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity.
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Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice.
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Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.
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N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.
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Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin contribute to cortical pathogenesis in HD mice.
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Huntingtin phosphorylation acts as a molecular switch for anterograde/retrograde transport in neurons.
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Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms.
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Hypothalamic huntingtin-associated protein 1 as a mediator of feeding behavior.
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Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity.
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J Cell Biol
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Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease.
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Transgenic rat model of Huntington's disease.
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Polyglutamine expansion of huntingtin impairs its nuclear export.
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Adenosine A(2A) receptor mediates microglial process retraction.
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Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice.
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J Cell Biol
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Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice.
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J Neurosci
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Polyglutamine domain modulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration.
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Nat Neurosci
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Differential activities of the ubiquitin-proteasome system in neurons versus glia may account for the preferential accumulation of misfolded proteins in neurons.
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J Neurosci
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Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease.
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Hum Mol Genet
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Pathological cell-cell interactions are necessary for striatal pathogenesis in a conditional mouse model of Huntington's disease.
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Mol Neurodegener
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Multiple pathways contribute to the pathogenesis of Huntington disease.
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Mol Neurodegener
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Lack of huntingtin-associated protein-1 causes neuronal death resembling hypothalamic degeneration in Huntington's disease.
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J Neurosci
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Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs.
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Hum Mol Genet
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Characterization of a brain-enriched chaperone, MRJ, that inhibits Huntingtin aggregation and toxicity independently.
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J Biol Chem
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Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice.
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J Biol Chem
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Regulation of intracellular trafficking of huntingtin-associated protein-1 is critical for TrkA protein levels and neurite outgrowth.
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J Neurosci
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Huntingtin-associated protein-1 is a modifier of the age-at-onset of Huntington's disease.
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Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin.
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Mutant huntingtin: nuclear translocation and cytotoxicity mediated by GAPDH.
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Huntingtin-associated protein 1 interacts with Ahi1 to regulate cerebellar and brainstem development in mice.
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Huntingtin and its role in neuronal degeneration.
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Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice.
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Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release.
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Hum Mol Genet
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TWEAK-Fn14 pathway inhibition protects the integrity of the neurovascular unit during cerebral ischemia.
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Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments.
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Neuronal expression of TATA box-binding protein containing expanded polyglutamine in knock-in mice reduces chaperone protein response by impairing the function of nuclear factor-Y transcription factor.
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Regulation of intracellular HAP1 trafficking.
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Polyglutamine expansion reduces the association of TATA-binding protein with DNA and induces DNA binding-independent neurotoxicity.
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Huntingtin-associated protein-1 interacts with pro-brain-derived neurotrophic factor and mediates its transport and release.
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Intracellular degradation of misfolded proteins in polyglutamine neurodegenerative diseases.
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Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation.
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Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates.
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AHI-1 interacts with BCR-ABL and modulates BCR-ABL transforming activity and imatinib response of CML stem/progenitor cells.
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Neuronal Abelson helper integration site-1 (Ahi1) deficiency in mice alters TrkB signaling with a depressive phenotype.
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Sex-dependent effect of BAG1 in ameliorating motor deficits of Huntington disease transgenic mice.
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Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A.
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Activation of gene transcription by heat shock protein 27 may contribute to its neuronal protection.
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Huntingtin-associated protein-1 deficiency in orexin-producing neurons impairs neuronal process extension and leads to abnormal behavior in mice.
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Immunohistochemical localization of huntingtin-associated protein 1 in endocrine system of the rat.
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Context-dependent dysregulation of transcription by mutant huntingtin.
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Polyglutamine toxicity in non-neuronal cells.
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Synaptic mutant huntingtin inhibits synapsin-1 phosphorylation and causes neurological symptoms.
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Nuclear accumulation of polyglutamine disease proteins and neuropathology.
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Transcriptional dysregulation of TrkA associates with neurodegeneration in spinocerebellar ataxia type 17.
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14-3-3 protein interacts with Huntingtin-associated protein 1 and regulates its trafficking.
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Impaired alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor trafficking and function by mutant huntingtin.
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J Biol Chem
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Longitudinal transcriptomic dysregulation in the peripheral blood of transgenic Huntington's disease monkeys.
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Loss of Ahi1 affects early development by impairing BM88/Cend1-mediated neuronal differentiation.
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J Neurosci
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Huntingtin-associated protein 1 regulates postnatal neurogenesis and neurotrophin receptor sorting.
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Brainstem Hap1-Ahi1 is involved in insulin-mediated feeding control.
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Loss of huntingtin-associated protein 1 impairs insulin secretion from pancreatic β-cells.
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Hypothalamic Ahi1 mediates feeding behavior through interaction with 5-HT2C receptor.
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TDP-43 causes differential pathology in neuronal versus glial cells in the mouse brain.
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Lack of interleukin-1 type 1 receptor enhances the accumulation of mutant huntingtin in the striatum and exacerbates the neurological phenotypes of Huntington's disease mice.
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A huntingtin-HAP1-PCM1 pathway in ciliogenesis.
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Thermal decomposition and kinetics studies on 1,4-dinitropiperazine (DNP).
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