483 results found
    1. Cell Biology

    Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases

    King Faisal Yambire et al.
    Transcription factors KLF2 and ETV1 repress the transcriptional program of mitochondrial biogenesis, resulting in impaired mitochondrial function in lysosomal storage diseases.
    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. Biochemistry and Chemical Biology
    2. Cell Biology

    Intracellular cholesterol trafficking is dependent upon NPC2 interaction with lysobisphosphatidic acid

    Leslie A McCauliff et al.
    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. 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. Cell Biology

    Intracellular sphingosine releases calcium from lysosomes

    Doris Höglinger et al.
    Sphingosine acts as a lipid messenger molecule in intracellular signaling networks that are relevant to lysosomal function and Niemann-Pick disease.
    1. Neuroscience

    Characterization of small fiber pathology in a mouse model of Fabry disease

    Lukas Hofmann et al.
    Globotriaosylcermide directly impacts neuronal integrity and ion channel function as potential mechanism underlying small fiber pathology in Fabry disease.
    1. Cell Biology
    2. Developmental Biology

    The tumor suppressor PTPRK promotes ZNRF3 internalization and is required for Wnt inhibition in the Spemann organizer

    Ling-Shih Chang et al.
    Two cancer related proteins, tyrosine phosphatase PTPRK and ubiquitin ligase ZNRF3, interact to downregulate Wnt receptors, thereby regulating Spemann organizer function during Xenopus development.
    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. Biochemistry and Chemical Biology
    2. Cell Biology

    The vacuolar-ATPase complex and assembly factors, TMEM199 and CCDC115, control HIF1α prolyl hydroxylation by regulating cellular iron levels

    Anna L Miles et al.
    An unbiased genome-wide human forward genetic screen identifies the vacuolar ATPase complex and assembly factors as regulators of HIF stability through their actions on intracellular iron metabolism.
    1. Developmental Biology
    2. Neuroscience

    Regulation of mitochondria-dynactin interaction and mitochondrial retrograde transport in axons

    Catherine M Drerup et al.
    Actr10, a dynactin pointed end protein, is essential for mitochondrial interaction with the dynein-dynactin retrograde motor and subsequent transport.

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