301 results found
    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. Developmental Biology
    2. Neuroscience

    APP interacts with LRP4 and agrin to coordinate the development of the neuromuscular junction in mice

    Hong Y Choi et al.
    Proteins implicated in Alzheimer’s disease, including amyloid precursor protein and ApoE receptors, interact with each other and with a signalling molecule called agrin to influence the development of the neuromuscular junction.
    1. Genetics and Genomics
    2. Medicine

    SMA-miRs (miR-181a-5p, -324-5p, and -451a) are overexpressed in spinal muscular atrophy skeletal muscle and serum samples

    Emanuela Abiusi et al.
    The identification of SMA-miR supports the pathogenic role of skeletal muscle in spinal muscular atrophy and, integrated in the SMA-score, may help to improve the phenotypic prediction for patients.
    1. Cell Biology
    2. Stem Cells and Regenerative Medicine

    Inducible depletion of adult skeletal muscle stem cells impairs the regeneration of neuromuscular junctions

    Wenxuan Liu et al.
    Skeletal muscle stem cells play important roles in the regeneration of neuromuscular junctions, and so present new targets for therapies to treat neuromuscular decline observed in the context of aging and various neuromuscular diseases.
    1. Developmental Biology
    2. Stem Cells and Regenerative Medicine

    A 3D culture model of innervated human skeletal muscle enables studies of the adult neuromuscular junction

    Mohsen Afshar Bakooshli et al.
    Human skeletal muscle progenitors and motor neurons self-organize in three-dimensional co-culture to form functional neuromuscular junctions that developmentally mature from the embryonic to the adult state.
    1. Neuroscience

    Dynamic neuromuscular remodeling precedes motor-unit loss in a mouse model of ALS

    Éric Martineau et al.
    Motor axons undergo dynamic branch-specific changes for weeks before complete neuronal degeneration in a model of amyotrophic lateral sclerosis, highlighting the importance of peripheral factors, intrinsic and extrinsic to motoneurons.
    1. Medicine

    Drug Discovery: From worms to fish to mice

    Guy M Benian, Hyojung J Choo
    An multi-species approach can be used to identify small molecules with properties that might prove useful for the treatment of some neuromuscular diseases.
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    1. Neuroscience

    Nemo-like kinase is a novel regulator of spinal and bulbar muscular atrophy

    Tiffany W Todd et al.
    Nemo-like kinase is a key protein promoting the pathogenesis of spinal and bulbar muscular atrophy.
    1. Structural Biology and Molecular Biophysics
    2. Cell Biology

    Structures of TorsinA and its disease-mutant complexed with an activator reveal the molecular basis for primary dystonia

    F Esra Demircioglu et al.
    High resolution structures of the essential human AAA+ ATPase TorsinA and its disease mutant in complex with an activator reveal details of the interaction that will guide drug design and further functional characterization.
    1. Cell Biology
    2. Developmental Biology

    Mouse retinal cell behaviour in space and time using light sheet fluorescence microscopy

    Claudia Prahst et al.
    Improved 3D and 4D imaging of neurovascular processes across scales reveals new insights into eye disease mouse models and shows retinal vessels are significantly distorted using standard flat-mount confocal imaging.

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