24 results found
    1. Cell Biology
    2. Neuroscience

    Antagonistic roles for Ataxin-2 structured and disordered domains in RNP condensation

    Amanjot Singh et al.
    Ataxin-2 interacts with neuronal mRNA in RNP granules.
    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

    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. 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. 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. 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. 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. 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

    RNG105/caprin1, an RNA granule protein for dendritic mRNA localization, is essential for long-term memory formation

    Kei Nakayama et al.
    The formation of long-term memory in mice requires an element of RNA granules that localizes messenger RNAs to dendrites.
    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.

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