110 results found
    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. Cell Biology
    2. Neuroscience

    Presynaptic APP levels and synaptic homeostasis are regulated by Akt phosphorylation of huntingtin

    Julie Bruyère et al.
    Reducing Akt-mediated huntingtin phosphorylation decreases APP accumulation at the synapse by reducing its anterograde axonal transport and ameliorates learning and memory in a mouse model of familial Alzheimer 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. 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. 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.
    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. 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. Cell Biology
    2. Neuroscience

    Phagocytic glia are obligatory intermediates in transmission of mutant huntingtin aggregates across neuronal synapses

    Kirby M Donnelly et al.
    Prion-like transfer of mutant huntingtin aggregates from presynaptic to postsynaptic neurons is enhanced by neuronal silencing and requires passage through the cytoplasm of Draper-expressing phagocytic glia in adult Drosophila brains.
    1. 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.

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