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    1. Biochemistry and Chemical Biology
    2. Human Biology and Medicine

    The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains

    Erin G Conlon et al.
    The C9orf72 expansion binds and sequesters the splicing factor hnRNP H leading to insoluble G-quadruplex aggregates that functionally reduce hnRNP H, thereby producing splicing defects.
    1. Cell Biology
    2. Neuroscience

    C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import

    Lindsey R Hayes et al.
    Poly-PR and poly-GR interact with importin β, disrupt importin-cargo loading, and inhibit nuclear import in permeabilized cells in a manner that can be rescued by RNA.
    1. Cell Biology
    2. Neuroscience

    Implementation of an antibody characterization procedure and application to the major ALS/FTD disease gene C9ORF72

    Carl Laflamme et al.
    An easy-to-implement antibody validation pipeline addresses the reproducibility crisis resulting from the use of non-specific antibodies.
    1. Cell Biology

    Rab5 and Alsin regulate stress-activated cytoprotective signaling on mitochondria

    FoSheng Hsu et al.
    Oxidative stress leads to the translocation of Rab5 from endosome to mitochondria, regulated by ALS/Alsin, a component associated with amyotrophic lateral sclerosis, leading to mitochondrial-endosomal physical contacts and a cytoprotective response.
    1. Cell Biology
    2. Human Biology and Medicine

    Transcriptional activator TAp63 is upregulated in muscular atrophy during ALS and induces the pro-atrophic ubiquitin ligase Trim63

    Yannick von Grabowiecki et al.
    Atrophic muscles of patients and animal models developing amyotrophic lateral sclerosis show an upregulation of TAp63 that stimulates the expression of a pro-atrophic ubiquitin ligase.
    1. Structural Biology and Molecular Biophysics

    Structural Biology: NMR illuminates the pathways to ALS

    Tao Xie, Charalampos G Kalodimos
    Insight
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    1. Neuroscience

    Regeneration: Regrowing axons with alternative splicing

    Nicholas J Kramer, Aaron D Gitler
    Insight
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    1. Neuroscience

    Glycolysis upregulation is neuroprotective as a compensatory mechanism in ALS

    Ernesto Manzo et al.
    Degenerating motor neurons compensate for energetic deficits by upregulating glycolysis in flies and humans, with increased glucose availability improving locomotor function and lifespan in flies.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Cell-based HTS identifies a chemical chaperone for preventing ER protein aggregation and proteotoxicity

    Keisuke Kitakaze et al.
    Cell-based high-throughput screening identifies IBT21 as a chemical chaperone, that inhibits ER protein aggregation and prevents the cell death caused by a proteotoxin, the aggregation-prone prion protein.