4,534 results found
    1. Neuroscience

    Early dysfunction and progressive degeneration of the subthalamic nucleus in mouse models of Huntington's disease

    Jeremy F Atherton et al.
    In mouse models of Huntington's disease, the subthalamic nucleus, which suppresses movements, also exhibits impaired glutamate homeostasis, NMDA receptor-dependent mitochondrial oxidant stress, firing disruption, and 30% neuronal loss.
    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. Biochemistry and Chemical Biology
    2. Neuroscience

    eIF2B activator prevents neurological defects caused by a chronic integrated stress response

    Yao Liang Wong et al.
    Boosting the function of translation factor eIF2B by chronic small molecule administration prevents pathology in a neurodegenerative model of Vanishing White Matter disease characterized by a maladaptive stress response.
    1. Human Biology and Medicine
    2. 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. Cancer Biology
    2. Human Biology and Medicine

    The selective estrogen receptor downregulator GDC-0810 is efficacious in diverse models of ER+ breast cancer

    James D Joseph et al.
    GDC-0810 is a novel, orally bioavailable SERD that exhibits robust pre-clinical activity in models of ER+ breast cancer, including models of tamoxifen resistance, and those that express the ERα mutations, ER.Y537S and ER.D538G.
    1. Neuroscience

    A prediction model of working memory across health and psychiatric disease using whole-brain functional connectivity

    Masahiro Yamashita et al.
    Human neuroimaging and machine learning reveals a generalizable relationship between brain connectivity and working memory ability across healthy populations and distinct psychiatric diagnoses.
    1. Neuroscience

    Restoration of Mecp2 expression in GABAergic neurons is sufficient to rescue multiple disease features in a mouse model of Rett syndrome

    Kerstin Ure et al.
    Genetically restoring Mecp2 expression only in GABAergic neurons in a mouse model of Rett syndrome improves inhibitory signaling, extends lifespan and rescues most but not all behavioral deficits.
    1. Cell Biology
    2. Neuroscience

    FcγRIIb-SHIP2 axis links Aβ to tau pathology by disrupting phosphoinositide metabolism in Alzheimer's disease model

    Tae-In Kam et al.
    By binding to Fc gamma receptor IIb, amyloid beta induces a series of phosphorylation events that mediate the damaging effects of hyperphosphorylated tau proteins in Alzheimer's disease.
    1. Neuroscience

    Long-term adult human brain slice cultures as a model system to study human CNS circuitry and disease

    Niklas Schwarz et al.
    Viral transduction and gene manipulation of adult human brain slices will be of great value allowing investigations including therapeutic screening, electrophysiological and structural studies of properties of human CNS circuitry.

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