Normal huntingtin function: an alternative approach to Huntington's disease (Q28281904)

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

21 January 2018

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Differential loss of striatal projection neurons in Huntington disease

21 January 2018

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21 January 2018

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Modeling Huntington's disease in cells, flies, and mice

21 January 2018

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Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

21 January 2018

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The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease

21 January 2018

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RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

21 January 2018

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From target identification to drug screening assays for neurodegenerative diseases

21 January 2018

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Therapeutic opportunities in polyglutamine disease

21 January 2018

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Huntington's disease: new hope for therapeutics

21 January 2018

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Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

21 January 2018

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Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

21 January 2018

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Depletion of wild-type huntingtin in mouse models of neurologic diseases

21 January 2018

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Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

21 January 2018

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Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila

21 January 2018

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Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro

21 January 2018

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Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules

21 January 2018

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Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease

21 January 2018

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Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo

21 January 2018

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Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95.

21 January 2018

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Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease

21 January 2018

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Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

21 January 2018

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Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons

21 January 2018

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Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways

21 January 2018

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Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles

21 January 2018

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Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription

21 January 2018

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Huntington's disease: a synaptopathy?

21 January 2018

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Trinucleotide repeats and neurodegenerative disease.

21 January 2018

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Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

21 January 2018

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The hunt for huntingtin function: interaction partners tell many different stories

21 January 2018

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Huntingtin-protein interactions and the pathogenesis of Huntington's disease

21 January 2018

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A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease

21 January 2018

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SUMO modification of Huntingtin and Huntington's disease pathology

21 January 2018

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HEAT repeats in the Huntington's disease protein

21 January 2018

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HEAT repeats associated with condensins, cohesins, and other complexes involved in chromosome-related functions

21 January 2018

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The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor

21 January 2018

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Huntingtin contains a highly conserved nuclear export signal

21 January 2018

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Polyglutamine expansion of huntingtin impairs its nuclear export

21 January 2018

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Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

21 January 2018

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Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

21 January 2018

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Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells

21 January 2018

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Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus

21 January 2018

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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

21 January 2018

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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

21 January 2018

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Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

21 January 2018

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Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

21 January 2018

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A putative Drosophila homolog of the Huntington's disease gene

21 January 2018

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Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

21 January 2018

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Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme

21 January 2018

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The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.

21 January 2018

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Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo

21 January 2018

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Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity

21 January 2018

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Huntingtin interacting protein 1 induces apoptosis via a novel caspase-dependent death effector domain

21 January 2018

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Huntingtin-interacting protein HIP14 is a palmitoyl transferase involved in palmitoylation and trafficking of multiple neuronal proteins

21 January 2018

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Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2

21 January 2018

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Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells

21 January 2018

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New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins

21 January 2018

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Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

21 January 2018

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Inactivation of the mouse Huntington's disease gene homolog Hdh

21 January 2018

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Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

21 January 2018

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Homozygotes for Huntington's disease

21 January 2018

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Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat

21 January 2018

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Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion

21 January 2018

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The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin

21 January 2018

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Life without huntingtin: normal differentiation into functional neurons

21 January 2018

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Huntingtin is required for normal hematopoiesis

21 January 2018

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Wild-type huntingtin plays a role in brain development and neuronal survival

21 January 2018

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Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease

21 January 2018

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Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing

21 January 2018

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Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi

21 January 2018

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The serum- and glucocorticoid-induced kinase SGK inhibits mutant huntingtin-induced toxicity by phosphorylating serine 421 of huntingtin.

21 January 2018

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Neurotrophin-4/5 promotes survival and differentiation of rat striatal neurons developing in culture

21 January 2018

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Trophic and protective actions of brain-derived neurotrophic factor on striatal DARPP-32-containing neurons in vitro.

21 January 2018

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Co-localization of brain-derived neurotrophic factor (BDNF) and wild-type huntingtin in normal and quinolinic acid-lesioned rat brain

21 January 2018

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Anterograde transport of brain-derived neurotrophic factor and its role in the brain

21 January 2018

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Early striatal dendrite deficits followed by neuron loss with advanced age in the absence of anterograde cortical brain-derived neurotrophic factor.

21 January 2018

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Brain-derived neurotrophic factor-mediated protection of striatal neurons in an excitotoxic rat model of Huntington's disease, as demonstrated by adenoviral gene transfer

21 January 2018

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Brain-derived neurotrophic factor prevents changes in Bcl-2 family members and caspase-3 activation induced by excitotoxicity in the striatum.

21 January 2018

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A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

21 January 2018

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Differential usage of multiple brain-derived neurotrophic factor promoters in the rat brain following neuronal activation

21 January 2018

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Identification of brain-derived neurotrophic factor promoter regions mediating tissue-specific, axotomy-, and neuronal activity-induced expression in transgenic mice

21 January 2018

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Neuronal activity regulates the developmental expression and subcellular localization of cortical BDNF mRNA isoforms in vivo

21 January 2018

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Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

21 January 2018

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Fast transport and retrograde movement of huntingtin and HAP 1 in axons

21 January 2018

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Cargo-carrying motor vehicles on the neuronal highway: transport pathways and neurodegenerative disease.

21 January 2018

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Neuron-specific expression of the rat brain type II sodium channel gene is directed by upstream regulatory elements

21 January 2018

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Silencing the type II sodium channel gene: a model for neural-specific gene regulation

21 January 2018

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A common silencer element in the SCG10 and type II Na+ channel genes binds a factor present in nonneuronal cells but not in neuronal cells

21 January 2018

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REST: A mammalian silencer protein that restricts sodium channel gene expression to neurons

21 January 2018

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The neuron-restrictive silencer factor (NRSF): a coordinate repressor of multiple neuron-specific genes

21 January 2018

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Genome-wide analysis of repressor element 1 silencing transcription factor/neuron-restrictive silencing factor (REST/NRSF) target genes

21 January 2018

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Synaptic dysfunction in Huntington's disease: a new perspective.

21 January 2018

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Postsynaptic signaling and plasticity mechanisms

21 January 2018

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Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.

21 January 2018

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Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse

21 January 2018

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Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course

21 January 2018

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Progressive loss of BDNF in a mouse model of Huntington's disease and rescue by BDNF delivery

21 January 2018

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Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease

21 January 2018

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Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

21 January 2018

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Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice

21 January 2018

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Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice

21 January 2018

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Brain-derived neurotrophic factor in Huntington disease

21 January 2018

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Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism.

21 January 2018

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Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease.

21 January 2018

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Brain-derived neurotrophic factor modulates dopaminergic deficits in a transgenic mouse model of Huntington's disease

21 January 2018

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Mutant protein in Huntington disease is resistant to proteolysis in affected brain

21 January 2018

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Mutant huntingtin promotes the fibrillogenesis of wild-type huntingtin: a potential mechanism for loss of huntingtin function in Huntington's disease

21 January 2018

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Calcium-dependent cleavage of endogenous wild-type huntingtin in primary cortical neurons

21 January 2018

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Huntingtin is degraded to small fragments by calpain after ischemic injury

21 January 2018

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Increased GABAergic function in mouse models of Huntington's disease: reversal by BDNF.

21 January 2018

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Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis

21 January 2018

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Copper stimulates endocytosis of the prion protein

21 January 2018

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The prion protein and neuronal zinc homeostasis

21 January 2018

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21 January 2018

Copper and prion disease

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Signal transduction through prion protein

21 January 2018

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Cellular prion protein transduces neuroprotective signals

21 January 2018

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Prion protein is necessary for normal synaptic function

21 January 2018

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Post-natal knockout of prion protein alters hippocampal CA1 properties, but does not result in neurodegeneration

21 January 2018

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Cellular prion protein binds laminin and mediates neuritogenesis

21 January 2018

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Cellular prion protein inhibits proapoptotic Bax conformational change in human neurons and in breast carcinoma MCF-7 cells

21 January 2018

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Towards cellular receptors for prions

21 January 2018

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The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses

21 January 2018

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Comparative sequence analysis of the human and pufferfish Huntington's disease genes

21 January 2018

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Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's disease

21 January 2018

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Characterization of the Huntington's disease (HD) gene homologue in the zebrafish Danio rerio.

21 January 2018

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Neuronal degeneration in the basal ganglia and loss of pallido-subthalamic synapses in mice with targeted disruption of the Huntington's disease gene

21 January 2018

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Wild-type huntingtin protects from apoptosis upstream of caspase-3.

21 January 2018

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Wild-type huntingtin protects neurons from excitotoxicity

31 March 2020

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Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum.

31 March 2020

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Cellular localization of huntingtin in striatal and cortical neurons in rats: lack of correlation with neuronal vulnerability in Huntington's disease.

31 March 2020

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Calpain activation in Huntington's disease.

31 March 2020

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Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

31 March 2020

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Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

31 March 2020

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Neuropathological findings in Wolf-Hirschhorn (4p-) syndrome

31 March 2020

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Neurons lacking huntingtin differentially colonize brain and survive in chimeric mice

31 March 2020

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TrkB signaling is required for postnatal survival of CNS neurons and protects hippocampal and motor neurons from axotomy-induced cell death.

31 March 2020

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Expression of the striatal DARPP-32/ARPP-21 phenotype in GABAergic neurons requires neurotrophins in vivo and in vitro.

31 March 2020

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Synapsins as mediators of BDNF-enhanced neurotransmitter release

31 March 2020

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Brain-derived neurotrophic factor promotes differentiation of striatal GABAergic neurons

31 March 2020

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The neurotrophins BDNF, NT-3 and NT-4/5 promote survival and morphological and biochemical differentiation of striatal neurons in vitro

31 March 2020

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Expression of brain-derived neurotrophic factor in cortical neurons is regulated by striatal target area.

31 March 2020

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Neural expression of the Huntington's disease gene as a chordate evolutionary novelty.

31 March 2020

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