21 results found
    1. Neuroscience

    Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration

    Kuchuan Chen et al.
    In Drosophila, the loss of Frataxin causes iron accumulation in the nervous system, which in turn enhances sphingolipid synthesis and activation of PDK1 and Mef2, which leads to neurodegeneration.
    1. Neuroscience

    Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals

    Kuchuan Chen et al.
    The iron/sphingolipid/PDK1/Mef2 pathway is activated in mammals upon loss of Frataxin.
    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. Biochemistry and Chemical Biology

    Ataxin-1 oligomers induce local spread of pathology and decreasing them by passive immunization slows Spinocerebellar ataxia type 1 phenotypes

    Cristian A Lasagna-Reeves et al.
    Building on previous work (Lasagna-Reeves et al., 2015) it is shown that polyglutamine ATXN1 oligomers propagate locally in SCA1 mice, and that passive immunotherapy targeting soluble oligomers can lead to an improvement in motor coordination and a modest increase in life span.
    1. Neuroscience

    Inducible and reversible phenotypes in a novel mouse model of Friedreich’s Ataxia

    Vijayendran Chandran et al.
    Restoration of endogenous frataxin levels reverses neurologic and cardiac phenotypes associated with Friedreich's ataxia in adult mice even after significant motor dysfunction.
    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
    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.
    1. Structural Biology and Molecular Biophysics

    Ciliate mitoribosome illuminates evolutionary steps of mitochondrial translation

    Victor Tobiasson, Alexey Amunts
    The ciliate mitoribosome structure provides new insights into the diversity of translation and its evolution.
    1. Cell Biology

    Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology

    Peipei Zhang et al.
    OptoGranules reveal the function of G3BP1 as a stress granule scaffold and demonstrate that protracted stress granule assembly is sufficient to drive neurodegeneration and the evolution of ALS-FTD pathology.

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