53 results found
    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. Cell Biology

    Translation in amino-acid-poor environments is limited by tRNAGln charging

    Natalya N Pavlova et al.
    Amino acid limitation triggers preferential uncharging of glutamine-specific tRNAs and depletes polyglutamine tract-containing proteins from mammalian cells, revealing a potential role for polyglutamine tracts as sensors of glutamine sufficiency.
    1. Neuroscience

    Nemo-like kinase is a novel regulator of spinal and bulbar muscular atrophy

    Tiffany W Todd et al.
    Nemo-like kinase is a key protein promoting the pathogenesis of spinal and bulbar muscular atrophy.
    1. Cell Biology
    2. Neuroscience

    Ubiquitin-interacting motifs of ataxin-3 regulate its polyglutamine toxicity through Hsc70-4-dependent aggregation

    Sean L Johnson et al.
    Pathogenesis in Spinocerebellar Ataxia Type 3 is enhanced by the heat-shock protein family member, Hsc70-4, uncovering new mechanisms of toxicity for this disease and suggesting pleiotropic roles for chaperones.
    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. 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. 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. Neuroscience

    A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1

    Cristian A Lasagna-Reeves et al.
    Studies in a mouse model of spinocerebellar ataxia type 1 show that a protein called capicua stabilizes toxic ataxin-1 oligomers offering a possible explanation for regional patterns of neurodegeneration.
    1. Genetics and Genomics

    miR-1 coordinately regulates lysosomal v-ATPase and biogenesis to impact proteotoxicity and muscle function during aging

    Isabelle Schiffer et al.
    Coordinate control of lysosomal v-ATPase subunits and biogenesis factor TFEB by miR-1 identifies a key regulatory axis with strong links to age-related proteotoxic disease.
    1. Cell Biology
    2. Neuroscience

    Ataxin-7 and Non-stop coordinate SCAR protein levels, subcellular localization, and actin cytoskeleton organization

    Veronica Cloud et al.
    An unbiased biochemical screen reveals a direct link between gene regulation and actin cytoskeleton remodeling.

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