18 results found
    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. Neuroscience

    Regeneration: Regrowing axons with alternative splicing

    Nicholas J Kramer, Aaron D Gitler
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    1. Chromosomes and Gene Expression
    2. Human Biology and Medicine

    Mutations in L-type amino acid transporter-2 support SLC7A8 as a novel gene involved in age-related hearing loss

    Meritxell Espino Guarch et al.
    SLC7A8 a neutral amino acid transporter has a key role in the maintenance of hearing during aging and its absence causes early onset of hearing loss.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    The translation elongation factor eEF1A1 couples transcription to translation during heat shock response

    Maria Vera et al.
    Eukaryotic translation elongation factor 1A1 controls the process of heat shock response, from transcriptional activation of the HSP70 gene, to HSP70 mRNA stabilization, nuclear export, and translation.
    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

    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. Neuroscience

    The critical role of membralin in postnatal motor neuron survival and disease

    Bo Yang et al.
    Membralin mutant mice manifest a severe and early-onset motor neuron disease with defects in endoplasmic reticulum-associated protein degradation.
    1. Neuroscience

    Whole brain delivery of an instability-prone Mecp2 transgene improves behavioral and molecular pathological defects in mouse models of Rett syndrome

    Mirko Luoni et al.
    Global brain transduction of the instability-prone Mecp2 transgene by systemic AAV-PHP.eB administration is safe and effective in protecting male and female Mecp2 mutant mice from the Rett syndrome disease phenotype.
    1. Neuroscience

    Remodeling of lumbar motor circuitry remote to a thoracic spinal cord injury promotes locomotor recovery

    Ying Wang et al.
    Retrograde transport of NT-3 stimulated the reorganization of lumbar neural circuitry and synaptic connectivity remote to a thoracic SCI, along with improved behavioral recovery.
    1. Neuroscience

    Revealing a novel nociceptive network that links the subthalamic nucleus to pain processing

    Arnaud Pautrat et al.
    The subthalamic nucleus is linked to a nociceptive network and involved in nociceptive processing and perception.

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