142 results found
    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. 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
    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

    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. Biochemistry and Chemical Biology
    2. Medicine

    Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism

    Erin G Conlon et al.
    Idiopathic patients along a complex motor neuron disease-dementia spectrum exhibit mRNA splicing changes that are due to multi-protein insolubility.
    1. Neuroscience

    Neuromuscular Disease: Protecting the nerve terminals

    Jonathan D Glass
    Maintaining the connections between nerve cells and muscle could help to slow the progression of amyotrophic lateral sclerosis.
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    1. Biochemistry and Chemical Biology

    RNA-Binding Proteins: A matter of balance

    Aaron D Gitler, John D Fryer
    New analyses shift the view that some forms of amyotrophic lateral sclerosis and frontotemporal dementia are due to defects in a single RNA-binding protein.
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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. 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. 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.

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