50 results found
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    The cooperative binding of TDP-43 to GU-rich RNA repeats antagonizes TDP-43 aggregation

    Juan Carlos Rengifo-Gonzalez et al.
    The RNA-mediated higher order assembly of TDP-43, a protein associated with neurodegenerative diseases, preserves its solubility by reducing the risk of multivalent interactions between low complexity domains.
    1. Genetics and Genomics
    2. Neuroscience

    Translational control of polyamine metabolism by CNBP is required for Drosophila locomotor function

    Sonia Coni et al.
    A decrease of the type 2 myotonic dystrophy gene product CNBP causes an impairment of polyamine metabolism and locomotor dysfunction that can be reverted with polyamine supplementation.
    1. Biochemistry and Chemical Biology
    2. Neuroscience

    Multiple pathways of toxicity induced by C9orf72 dipeptide repeat aggregates and G4C2 RNA in a cellular model

    Frédéric Frottin et al.
    Protein aggregates resulting from mutations in C9orf72 impair different aspects of cellular quality control in the cytosol and the nucleus, but mRNA-mediated effects contribute more strongly to toxicity.
    1. Cell Biology
    2. Neuroscience

    Amphiphysin I cleavage by asparagine endopeptidase leads to tau hyperphosphorylation and synaptic dysfunction

    Xingyu Zhang et al.
    Amphiphysin I is identified as a new substrate of AEP, and the resultant fragment induces synaptic dysfunction and promotes tau hyperphosphorylation via activating CDK5 kinase.
    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.
    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. Neuroscience

    Seizures are a druggable mechanistic link between TBI and subsequent tauopathy

    Hadeel Alyenbaawi et al.
    A traumatic brain injury model is invented for larval zebrafish and applied to a new fluorescent 'tauopathy reporter fish', revealing a role for seizures in progression towards dementias.
    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

    Cytosolic calcium regulates cytoplasmic accumulation of TDP-43 through Calpain-A and Importin α3

    Jeong Hyang Park et al.
    Genetic and optogenetic analyses in Drosophila neurons reveal calcium as one of the key regulators of nucleocytoplasmic localization of TDP-43 via Calpain-A and Importin α3.

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