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    1. Neuroscience

    Preserving neuromuscular synapses in ALS by stimulating MuSK with a therapeutic agonist antibody

    Sarah Cantor et al.
    An agonist antibody to MuSK, delivered after disease onset, decreases the loss of neuromuscular synapses, improves motor function and extends the lifespan of ALS mice.
    1. Genetics and Genomics
    2. Neuroscience

    Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology

    Eric N Anderson et al.
    Traumatic injury leads to functional defects in nucleocytoplasmic transport and TDP-43 pathology in multiple model systems.
    1. Neuroscience

    Cytosolic calcium regulates cytoplasmic accumulation of TDP-43 through Calpain-A and Importin α3

    Jeong Hyang Park et al.
    Genetic and optogenetic analyses in Drosophila neurons reveal calcium as one of the key regulators of nucleocytoplasmic localization of TDP-43 via Calpain-A and Importin α3.
    1. Neuroscience

    Relaxation of synaptic inhibitory events as a compensatory mechanism in fetal SOD spinal motor networks

    Pascal Branchereau et al.
    Electrophysiological and simulation approaches show that a chloride-related longer relaxation of the inhibitory synaptic events partially compensates the early defect in the chloride homeostasis detected in fetal SOD spinal motoneurons.
    1. Biochemistry and Chemical Biology
    2. Neuroscience

    Multiple pathways of toxicity induced by C9orf72 dipeptide repeat aggregates and G4C2 RNA in a cellular model

    Frédéric Frottin et al.
    Protein aggregates resulting from mutations in C9orf72 impair different aspects of cellular quality control in the cytosol and the nucleus, but mRNA-mediated effects contribute more strongly to toxicity.
    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.
    1. Biochemistry and Chemical Biology

    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

    A novel ALS-associated variant in UBQLN4 regulates motor axon morphogenesis

    Brittany M Edens et al.
    A novel ALS-associated variant in UBQLN4 impairs proteasome function and beta-catenin degradation to drive aberrant axon morphogenesis in motor neurons.
    1. Structural Biology and Molecular Biophysics

    Thermal fluctuations of immature SOD1 lead to separate folding and misfolding pathways

    Ashok Sekhar et al.
    Solution NMR spectroscopy reveals the energy landscape of immature Cu, Zn-superoxide dismutase and leads to atomic resolution structural models of transiently populated non-native oligomers.
    1. Neuroscience

    Enhanced insulin signalling ameliorates C9orf72 hexanucleotide repeat expansion toxicity in Drosophila

    Magda L Atilano et al.
    Modulation of insulin signalling could be an effective therapeutic approach against hexanucleotide repeat expansion related to c9ALS/FTD neurodegenerative diseases.