119 results found
    1. Cell Biology

    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. 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. Medicine
    2. Neuroscience

    Neural population dynamics in human motor cortex during movements in people with ALS

    Chethan Pandarinath et al.
    Neural ensemble activity in the human motor cortex contains dynamical structure that is independent of movement parameters and is not well-explained by current models.
    1. Neuroscience

    Early intrinsic hyperexcitability does not contribute to motoneuron degeneration in amyotrophic lateral sclerosis

    Félix Leroy et al.
    Contrary to a long-standing hypothesis, the neuronal death that leads to muscle wastage in amyotrophic lateral sclerosis does not result from overactivity of those neurons during development.
    1. Neuroscience

    Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106

    Barbara Celona et al.
    Zfp106 functions as an RNA binding protein, binds directly to GGGGCC RNA repeats, is required in motor neurons to prevent ALS-like neurodegeneration in mice, and can suppress neurotoxicity in an established fly model of ALS.
    1. Cell Biology
    2. Neuroscience

    TFEB/Mitf links impaired nuclear import to autophagolysosomal dysfunction in C9-ALS

    Kathleen M Cunningham et al.
    Impaired nuclear import of the transcription factor TFEB/MITF is a major cause of autophagy and lysosome dysfunction in amyotrophic lateral sclerosis caused by mutations in the C9orf72 gene.
    1. Neuroscience

    Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis

    Shuo-Chien Ling et al.
    Elevating FUS level by saturating autoregulation loop in mice causes aggressive motor neuron disease via disrupting protein and RNA homeostasis.
    1. Neuroscience

    Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS

    María de Lourdes Martínez-Silva et al.
    The most vulnerable motor units lose a fundamental firing property before the denervation of their muscle fibers in ALS mice, changing our view of the role of excitability in neurodegeneration.
    1. Cell Biology

    Nuclear export of misfolded SOD1 mediated by a normally buried NES-like sequence reduces proteotoxicity in the nucleus

    Yongwang Zhong et al.
    Cellular and genetic approaches reveal that exposure of a normally buried nuclear export signal (NES)-like sequence mediates export of ALS-linked mutant and misfolded wild-type SOD1 to the cytoplasm by CRM1.
    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.

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