876 results found
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    The lysosomal transporter MFSD1 is essential for liver homeostasis and critically depends on its accessory subunit GLMP

    David Massa López et al.
    The so far uncharacterized lysosomal transporter protein MFSD1 is essential for liver homeostasis and needs the highly glycosylated GLMP protein as an accessory subunit for stability.
    1. Neuroscience

    Agonist-specific voltage-dependent gating of lysosomal two-pore Na+ channels

    Xiaoli Zhang et al.
    Tricyclic antidepressants activate lysosomal two-pore Na+ channels in a voltage-dependent manner.
    1. Genetics and Genomics

    miR-1 coordinately regulates lysosomal v-ATPase and biogenesis to impact proteotoxicity and muscle function during aging

    Isabelle Schiffer et al.
    Coordinate control of lysosomal v-ATPase subunits and biogenesis factor TFEB by miR-1 identifies a key regulatory axis with strong links to age-related proteotoxic disease.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Lysosomal membrane glycoproteins bind cholesterol and contribute to lysosomal cholesterol export

    Jian Li, Suzanne R Pfeffer
    LAMP proteins, the major glycoproteins of the lysosome membrane, bind cholesterol directly and specifically, and interact with NPC1 and NPC2 proteins as part of the lysosomal cholesterol export process.
    1. Cell Biology

    High lumenal chloride in the lysosome is critical for lysosome function

    Kasturi Chakraborty et al.
    Lysosomes are highly enriched in chloride, which is essential for their degradative function.
    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. Neuroscience

    Peroxisomal dysfunctions cause lysosomal storage and axonal Kv1 channel redistribution in peripheral neuropathy

    Sandra Kleinecke et al.
    Dysfunctions of myelin peroxisomes cause a lysosomal storage-like disorder associated with alterations in glial and axonal membranes, which is the likely cause of nerve impairment in peroxisomal disorders.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Identification of NPC1 as the target of U18666A, an inhibitor of lysosomal cholesterol export and Ebola infection

    Feiran Lu et al.
    A nanomolar inhibitor of cholesterol transport out of endosomes/lysosomes can be crosslinked to the “sterol-sensing domain” of NPC1, which implicates this domain in the transmembrane transport of cholesterol.
    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

    The vacuole/lysosome is required for cell-cycle progression

    Yui Jin, Lois S Weisman
    The endo-lysosomal system plays a positive essential role in the initiation of the cell cycle in yeast.

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