118 results found
    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. 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. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    TRiC’s tricks inhibit huntingtin aggregation

    Sarah H Shahmoradian et al.
    Cryo-electron tomography reveals how a chaperone protein called TRiC reduces the ability of pathogenic mutant huntingtin proteins to form aggregates.
    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. 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. Structural Biology and Molecular Biophysics
    2. Chromosomes and Gene Expression

    Real-time imaging of Huntingtin aggregates diverting target search and gene transcription

    Li Li et al.
    Live imaging captures dynamics of Huntingtin aggregates plaguing the genome and waylaying target search.
    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.

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