508 results found
    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

    TTBK2 and primary cilia are essential for the connectivity and survival of cerebellar Purkinje neurons

    Emily Bowie, Sarah C Goetz
    Signaling at the primary cilium is important to sustain the morphology, connectivity, and survival of a key neural population within the brain.
    1. Cell Biology

    Site-specific phosphorylation and caspase cleavage of GFAP are new markers of Alexander disease severity

    Rachel A Battaglia et al.
    GFAP protein carries a distinct post-translational modification signature that facilitates its pathogenic accumulation and aggregation in astrocytes of patients who succumb to Alexander Disease very early in life.
    1. Cell Biology

    GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function

    Alondra Schweizer Burguete et al.
    Expanded repeat RNAs associated with human neurodegenerative diseases can become incorporated into transported granules in neurons, perturbing their function to cause neuritic branching defects.
    1. Structural Biology and Molecular Biophysics
    2. Chromosomes and Gene Expression

    Real-time imaging of Huntingtin aggregates diverting target search and gene transcription

    Li Li et al.
    Live imaging captures dynamics of Huntingtin aggregates plaguing the genome and waylaying target search.
    1. Neuroscience

    A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity

    Yuwei Yao et al.
    A cell-surface receptor called Gpr52 is able to lower the levels of the disease-causing protein mutant huntingtin and suppress its toxicity when knocked-down, making this receptor a promising drug target in Huntington's disease.
    1. Cell Biology
    2. Neuroscience

    Antagonistic roles for Ataxin-2 structured and disordered domains in RNP condensation

    Amanjot Singh et al.
    Ataxin-2 interacts with neuronal mRNA in RNP granules.
    1. Neuroscience

    Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis

    Benedikt Grünewald et al.
    Impaired GABAergic and glutamatergic synaptic function and loss of interneurons in the amygdala, hippocampus, and cerebellum cause characteristic disease symptoms in a mouse model juvenile neuronal ceroid lipofuscinosis.
    1. Cell Biology

    Human VPS13A is associated with multiple organelles and influences mitochondrial morphology and lipid droplet motility

    Wondwossen M Yeshaw et al.
    VPS13A is associated with the endoplasmic reticulum, mitochondria and lipid droplets and is required for cellular processes that require interaction between these organells.
    1. Neuroscience

    Rbfox1 up-regulation impairs BDNF-dependent hippocampal LTP by dysregulating TrkB isoform expression levels

    Francesco Tomassoni-Ardori et al.
    RbFox1 upregulation modulates the expression of hundreds of genes but only dysregulation of the TrkB.T1 receptor isoform expression causes BDNF-dependent LTP impairments.

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