555 results found
    1. Cell Biology

    Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases

    King Faisal Yambire, Lorena Fernandez-Mosquera ... Nuno Raimundo
    Transcription factors KLF2 and ETV1 repress the transcriptional program of mitochondrial biogenesis, resulting in impaired mitochondrial function in lysosomal storage diseases.
    1. Cell Biology

    Novel autophagy inducers by accelerating lysosomal clustering against Parkinson’s disease

    Yuki Date, Yukiko Sasazawa ... Shinji Saiki
    Lysosomal clustering around the microtubule-organizing centre would be a promising therapeutic strategy for Parkinson's disease.
    1. Cell Biology

    High lumenal chloride in the lysosome is critical for lysosome function

    Kasturi Chakraborty, KaHo Leung, Yamuna Krishnan
    Lysosomes are highly enriched in chloride, which is essential for their degradative function.
    1. Cell Biology
    2. Neuroscience

    Unraveling the link between neuropathy target esterase NTE/SWS, lysosomal storage diseases, inflammation, abnormal fatty acid metabolism, and leaky brain barrier

    Mariana I Tsap, Andriy S Yatsenko ... Halyna R Shcherbata
    Drosophila model reveals that NTE/SWS-associated neurodegeneration is a lysosomal storage disorder accompanied by a leaky brain permeability barrier, abnormal fatty acid metabolism, and inflammation.
    1. Cell Biology

    Rab12 is a regulator of LRRK2 and its activation by damaged lysosomes

    Xiang Wang, Vitaliy V Bondar ... Anastasia G Henry
    Rab12 was identified as a key modulator of LRRK2-dependent Rab phosphorylation that mediates the recruitment of LRRK2 to lysosomes in response to damage.
    1. Cell Biology
    2. Neuroscience

    VCP-dependent muscle degeneration is linked to defects in a dynamic tubular lysosomal network in vivo

    Alyssa E Johnson, Huidy Shu ... Graeme W Davis
    The AAA-ATPase VCP sustains sarcoplasmic proteostasis, in part, by controlling autophagosome-lysosome fusion and the integrity of a dynamic tubular lysosomal network.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Intracellular cholesterol trafficking is dependent upon NPC2 interaction with lysobisphosphatidic acid

    Leslie A McCauliff, Annette Langan ... Judith Storch
    Sterol kinetics and cell-based assays reveal a heretofore unknown step in cholesterol trafficking through the endolysosomal compartment, involving a direct functional interaction between NPC2 and lysosbisphosphatidic acid.
    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, Melanie Thelen ... Markus Damme
    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. Cell Biology

    Impaired lysosomal acidification triggers iron deficiency and inflammation in vivo

    King Faisal Yambire, Christine Rostosky ... Nuno Raimundo
    Impaired lysosomal acidification results in retention of iron inside lysosomes, triggering functional iron deficiency, dysfunctional mitochondria (especially mtDNA loss), and inflammation in vivo in a mouse model of lysosomal disease.
    1. Cell Biology

    Intracellular sphingosine releases calcium from lysosomes

    Doris Höglinger, Per Haberkant ... Carsten Schultz
    Sphingosine acts as a lipid messenger molecule in intracellular signaling networks that are relevant to lysosomal function and Niemann-Pick disease.

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