7 results found
    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 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. Cell Biology

    Mitochondria: Selective protein degradation ensures cellular longevity

    Sandra Malmgren Hill, Thomas Nyström
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    1. Cell Biology
    2. Neuroscience

    TTBK2 and primary cilia are essential for the connectivity and survival of cerebellar Purkinje neurons

    Emily Bowie, Sarah C Goetz
    Signaling at the primary cilium is important to sustain the morphology, connectivity, and survival of a key neural population within the brain.
    1. Cell Biology

    GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function

    Alondra Schweizer Burguete et al.
    Expanded repeat RNAs associated with human neurodegenerative diseases can become incorporated into transported granules in neurons, perturbing their function to cause neuritic branching defects.

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