90 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. 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. 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. Human Biology and Medicine
    2. Neuroscience

    A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity

    Yuwei Yao et al.
    A cell-surface receptor called Gpr52 is able to lower the levels of the disease-causing protein mutant huntingtin and suppress its toxicity when knocked-down, making this receptor a promising drug target in Huntington's disease.
    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.
    1. Neuroscience

    Mutant huntingtin enhances activation of dendritic Kv4 K+ channels in striatal spiny projection neurons

    Luis Carrillo-Reid et al.
    In mouse models of Huntington's disease, striatal spiny projection neurons up-regulate dendritic potassium channels, which impairs their normal function, but a zinc finger gene therapy can reverse this deficit.
    1. Cell Biology

    Spatial sequestration and detoxification of Huntingtin by the ribosome quality control complex

    Junsheng Yang et al.
    A genome-wide screen in yeast reveals that key proteins in ribosome quality control also regulate mutant Huntingtin aggregation and toxicity.
    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. Human Biology and Medicine
    2. Neuroscience

    Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein

    Ismael Al-Ramahi et al.
    This work validates PIP4K gamma as pharmacological target to ameliorate Huntington's disease.
  1. Plain-language Summaries of Research: The value of a healthy relationship

    Bridget M Kuehn
    Biomedical science benefits when plain language allows patients to engage with all stages of the research process.

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