332 results found
    1. Cell Biology
    2. Neuroscience

    The interactome of the copper transporter ATP7A belongs to a network of neurodevelopmental and neurodegeneration factors

    Heather S Comstra et al.
    The ATP7A interactome is the first comprehensive view of putative Menkes disease neurodegeneration mechanisms.
    1. Neuroscience

    Rigid firing sequences undermine spatial memory codes in a neurodegenerative mouse model

    Jingheng Cheng, Daoyun Ji
    In a mouse model of neurodegeneration, hippocampal firing sequences that normally encode spatial memories become disengaged from external space.
    1. Neuroscience

    Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration

    Kuchuan Chen et al.
    In Drosophila, the loss of Frataxin causes iron accumulation in the nervous system, which in turn enhances sphingolipid synthesis and activation of PDK1 and Mef2, which leads to neurodegeneration.
    1. Biophysics and Structural Biology
    2. Neuroscience

    Neurodegenerative disease mutations in TREM2 reveal a functional surface and distinct loss-of-function mechanisms

    Daniel L Kober et al.
    Structural, biophysical, and functional analyses reveal that mutations in TREM2 trigger either misfolding or reduced binding to cell-surface glycosaminoglycans, which segregate with neurodegenerative disease link and highlight a functional surface linked to the pathogenesis of Alzheimer's disease.
    1. Genes and Chromosomes
    2. Neuroscience

    Activation of GCN2 kinase by ribosome stalling links translation elongation with translation initiation

    Ryuta Ishimura et al.
    Activation of the integrated stress response by stalled translation elongation complexes attenuates neurodegeneration, and demonstrates a protective link between a decrease in the rate of translation initiation and defects in translation elongation.
    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. Cell Biology

    Stress-induced Cdk5 activity enhances cytoprotective basal autophagy in Drosophila melanogaster by phosphorylating acinus at serine437

    Nilay Nandi et al.
    Cdk5-mediated stabilization of Acinus in postmitotic neurons promotes autophagy and the removal of protein aggregates linked to neurodegeneration in Drosophila melanogaster.
    1. Cell Biology

    The GARP complex is required for cellular sphingolipid homeostasis

    Florian Fröhlich et al.
    The retrograde vesicular trafficking GARP complex, which is mutated in a neurodegenerative disease, is important for sphingolipid homeostasis in yeast and mammalian cells.
    1. Human Biology and Medicine
    2. Neuroscience

    Early tissue damage and microstructural reorganization predict disease severity in experimental epilepsy

    Philipp Janz et al.
    Non-invasive imaging of hippocampal neurodegeneration and structural reorganization during epileptogenesis allows the prediction of disease severity in mesial temporal lobe epilepsy, which may facilitate the early pharmacological intervention before seizure onset.
    1. Cell Biology
    2. Neuroscience

    Dual leucine zipper kinase-dependent PERK activation contributes to neuronal degeneration following insult

    Martin Larhammar et al.
    Two seemingly distinct cellular stress response pathways that contribute to neurodegeneration after axonal insults are now revealed to be under the control of a single master regulator of the neuronal injury response, the kinase DLK.

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