84 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. 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
    2. Human Biology and Medicine

    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

    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. Neuroscience

    Amyotrophic Lateral Sclerosis: Marking the differences in motoneurons

    Simon A Sharples, Patrick J Whelan
    A subgroup of the neurons that control muscles becomes less excitable shortly before the symptoms of ALS develop.
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    1. Biochemistry and Chemical Biology
    2. Human Biology and 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. Cell Biology
    2. Neuroscience

    VCP-dependent muscle degeneration is linked to defects in a dynamic tubular lysosomal network in vivo

    Alyssa E Johnson et al.
    The AAA-ATPase VCP sustains sarcoplasmic proteostasis, in part, by controlling autophagosome-lysosome fusion and the integrity of a dynamic tubular lysosomal network.
    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

    Stage-dependent remodeling of projections to motor cortex in ALS mouse model revealed by a new variant retrograde-AAV9

    Barbara Commisso et al.
    The projections from discrete areas to motor cortex increase over disease course in motoneuron disease model with selective spatial and temporal patterns.
    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.

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