244 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. Developmental Biology

    Autophagic cell death is dependent on lysosomal membrane permeability through Bax and Bak

    Jason Karch et al.
    Molecular pathways controlling autophagic cell death are regulated at the level of the lysosome through the activity of the pro-death Bcl-2 family member Bax/Bak.
    1. Cell Biology
    2. Developmental Biology

    Genetic screen in Drosophila muscle identifies autophagy-mediated T-tubule remodeling and a Rab2 role in autophagy

    Naonobu Fujita et al.
    Analyses of a developmentally regulated Drosophila myofiber remodeling program provide insight into induced autophagy required for T-tubule membrane reorganization, and uncover a conserved Rab2 role in autophagosome-lysosome fusion.
    1. Cell Biology
    2. Neuroscience

    Degradation of engulfed mitochondria is rate-limiting in Optineurin-mediated mitophagy in neurons

    Chantell S Evans, Erika LF Holzbaur
    In neuronal mitophagy, Parkin and OPTN induce efficient sequestration of damaged somal mitochondria into autophagosomes, but slow turnover via lysosomal acidification may be a point of vulnerability for the cell.
    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. Cell Biology

    Autophagy-dependent ribosomal RNA degradation is essential for maintaining nucleotide homeostasis during C. elegans development

    Yubing Liu et al.
    Embryonic development requires autophagy-dependent degradation of ribosomal RNAs.
    1. Cell Biology

    Selective sorting and destruction of mitochondrial membrane proteins in aged yeast

    Adam L Hughes et al.
    A new mitochondrial protein degradation pathway selectively sorts and destroys a subset of the mitochondrial membrane proteome to protect mitochondria in times of stress.
    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. Immunology and Inflammation
    2. Neuroscience

    Differential accumulation of storage bodies with aging defines discrete subsets of microglia in the healthy brain

    Jeremy Carlos Burns et al.
    Two novel subsets of microglia identified by their unique autofluorescence profiles differ in their subcellular organization, proteomic signatures and in their response to aging and lysosomal dysfunction.
    1. Neuroscience

    Parkin contributes to synaptic vesicle autophagy in Bassoon-deficient mice

    Sheila Hoffmann-Conaway et al.
    The presynaptic scaffolding protein Bassoon is involved in regulating neurotransmitter release by controlling synaptic vesicle pool size and vesicular protein turnover through increased ubiquitination and Parkin-dependent autophagy.

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