49 results found
    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. Cell Biology
    2. Neuroscience

    Implementation of an antibody characterization procedure and application to the major ALS/FTD disease gene C9ORF72

    Carl Laflamme et al.
    An easy-to-implement antibody validation pipeline addresses the reproducibility crisis resulting from the use of non-specific antibodies.
    1. Neuroscience

    Enhanced insulin signalling ameliorates C9orf72 hexanucleotide repeat expansion toxicity in Drosophila

    Magda L Atilano et al.
    Modulation of insulin signalling could be an effective therapeutic approach against hexanucleotide repeat expansion related to c9ALS/FTD neurodegenerative diseases.
    1. Cell Biology
    2. Neuroscience

    Antagonistic roles for Ataxin-2 structured and disordered domains in RNP condensation

    Amanjot Singh et al.
    Ataxin-2 interacts with neuronal mRNA in RNP granules.
    1. Cell Biology
    2. Neuroscience

    Frontotemporal dementia mutant Tau promotes aberrant Fyn nanoclustering in hippocampal dendritic spines

    Pranesh Padmanabhan et al.
    Super-resolution imaging reveals that the microtubule-associated protein Tau regulates the Fyn kinase organisation in dendrites, and that the frontotemporal dementia mutant Tau promotes aberrant Fyn clustering, potentially leading to synaptic dysfunction.
    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

    Role of the visual experience-dependent nascent proteome in neuronal plasticity

    Han-Hsuan Liu et al.
    A screen of visual-experience induced changes in newly-synthesized proteins in Xenopus optic tectum identifies unexpected candidate plasticity proteins.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Multiplex image-based autophagy RNAi screening identifies SMCR8 as ULK1 kinase activity and gene expression regulator

    Jennifer Jung et al.
    An image-based multiplex autophagosome RNAi screen targeting all Rab GTPases as well as their GAPs and GEFs identifies the Rab GEF SMCR8 as multifaceted autophagy modulator, which regulates kinase activity and gene expression of ULK1.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Pharmacological dimerization and activation of the exchange factor eIF2B antagonizes the integrated stress response

    Carmela Sidrauski et al.
    A drug-like molecule called ISRIB, which activates the translation initiation factor eIF2B, antagonizes stress responses as diverse as protein misfolding and nutrient deprivation, and restores protein synthesis, enhancing memory.
    1. Genetics and Genomics
    2. Neuroscience

    Integrated transcriptomic and neuroimaging brain model decodes biological mechanisms in aging and Alzheimer’s disease

    Quadri Adewale et al.
    Identification of causal genes and their effects on other biological determinants untangles the complexities of aging and Alzheimer's and can facilitate drug discovery for sustaining healthy aging and treating Alzheimer's.

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