171 results found
    1. Neuroscience

    Metformin reverses early cortical network dysfunction and behavior changes in Huntington’s disease

    Isabelle Arnoux et al.
    In a premanifest mouse model of Huntington's disease at a stage very far from disease onset, significant network and behavior dysregulation was found, being rebalanced by treatment with metformin.
    1. Genetics and Genomics
    2. Neuroscience

    Histone deacetylase knockouts modify transcription, CAG instability and nuclear pathology in Huntington disease mice

    Marina Kovalenko et al.
    Genetic knockout of Hdac2 modifies molecular and cellular phenotypes in Huntington’s disease mice and has a prominent transcriptional regulatory role in adult medium spiny neurons.
    1. Computational and Systems Biology
    2. Genetics and Genomics

    Downregulation of glial genes involved in synaptic function mitigates Huntington's disease pathogenesis

    Tarik Seref Onur et al.
    Cross-species transcriptomic analysis and high-throughput behavioral assays in a Drosophila model of Huntington's disease show that downregulation of glial genes involved in synaptic function compensates for disease-related excitotoxicity.
    1. Neuroscience

    Modulation of dopamine D1 receptors via histamine H3 receptors is a novel therapeutic target for Huntington's disease

    David Moreno-Delgado et al.
    Progression of Huntington's disease can be slowed by altering dopamine signalling through the Dopamine 1 receptor - Histamine 3 receptor heteromer.
    1. Genetics and Genomics
    2. Neuroscience

    Propensity for somatic expansion increases over the course of life in Huntington disease

    Radhia Kacher et al.
    Somatic instability of the CAG repeat increases progressively with age and disease progression in Huntington disease mutation carriers, starting with low levels in fetal brain tissues.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract

    Koning Shen et al.
    The polyQ tract of pathogenic Huntingtin causes aggregation when expanded in Huntington’s disease, but its two flanking domains control its conformational landscape, proteostasis and neurotoxicity.
    1. Neuroscience

    M2 cortex-dorsolateral striatum stimulation reverses motor symptoms and synaptic deficits in Huntington’s disease

    Sara Fernández-García et al.
    M2 cortex-dorsolateral striatum circuit is functionally altered in Huntington's disease and, by boosting its activity, we reverse symptoms at behavioral, physiological, and morphological level in symptomatic mice.
    1. Neuroscience

    Early dysfunction and progressive degeneration of the subthalamic nucleus in mouse models of Huntington's disease

    Jeremy F Atherton et al.
    In mouse models of Huntington's disease, the subthalamic nucleus, which suppresses movements, also exhibits impaired glutamate homeostasis, NMDA receptor-dependent mitochondrial oxidant stress, firing disruption, and 30% neuronal loss.
    1. Structural Biology and Molecular Biophysics
    2. Neuroscience

    Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function

    Ravi Vijayvargia et al.
    Structural and biochemical analysis of full-length huntingtin protein illuminates the impact of the polyglutamine region on its structure and function.
    1. Neuroscience

    Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription

    Rui Gao et al.
    Polyglutamine expansion in mutant huntingtin disrupts a novel transcription-coupled DNA repair complex, providing an undescribed mechanism of neuronal toxicity and degeneration in Huntington's disease.

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