51 results found
    1. Developmental Biology

    Straightjacket/α2δ3 deregulation is associated with cardiac conduction defects in myotonic dystrophy type 1

    Emilie Auxerre-Plantié et al.
    Drosophila DM1 models suggest that elevated cardiac expression of straightjacket/α2δ3, a regulatory subunit of voltage-gated calcium channel, contributes to cardiac conduction defects in DM1.
    1. Cell Biology
    2. Neuroscience

    EFHC1, implicated in juvenile myoclonic epilepsy, functions at the cilium and synapse to modulate dopamine signaling

    Catrina M Loucks et al.
    C. elegans EFHC1 influences neuronal transmission through the cilium and the synapse.
    1. Neuroscience

    Acute cerebellar knockdown of Sgce reproduces salient features of myoclonus-dystonia (DYT11) in mice

    Samantha Washburn et al.
    Acute knock down of Sgce that is mutated in Myoclonus Dystonia (DYT11), in the cerebellum of mice, leads to dystonia and myoclonus-like motor signs that like patients improve with alcohol.
    1. Neuroscience

    Corollary discharge in precerebellar nuclei of sleeping infant rats

    Didhiti Mukherjee et al.
    Infant rats distinguish between self- and other-generated movements, and they do so primarily when moving during active sleep.
    1. Genetics and Genomics
    2. Neuroscience

    Translational control of polyamine metabolism by CNBP is required for Drosophila locomotor function

    Sonia Coni et al.
    A decrease of the type 2 myotonic dystrophy gene product CNBP causes an impairment of polyamine metabolism and locomotor dysfunction that can be reverted with polyamine supplementation.
    1. Neuroscience

    Prenatal methadone exposure disrupts behavioral development and alters motor neuron intrinsic properties and local circuitry

    Gregory G Grecco et al.
    A novel, translational mouse model of prenatal methadone exposure was developed and a longitudinal assessment of offspring revealed impairments in physical, behavioral, and neuronal development.
    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. Neuroscience

    A transient postnatal quiescent period precedes emergence of mature cortical dynamics

    Soledad Domínguez et al.
    Maturation of cortical network dynamics is characterized by a nadir that signals the shift from local, loosely correlated, prominently sensory-driven patterns to internally organized, spatially distributed, and temporally precise activity.
    1. Neuroscience

    Developmental 'awakening' of primary motor cortex to the sensory consequences of movement

    James C Dooley, Mark S Blumberg
    Early in development, before neurons in primary motor cortex are involved in motor control, they undergo a rapid transition in how they process sensory information following sleep and wake movements.
    1. Neuroscience

    Defined neuronal populations drive fatal phenotype in a mouse model of Leigh syndrome

    Irene Bolea et al.
    Glutamatergic brainstem neurons drive motor and respiratory deficits, and GABAergic basal ganglia neurons cause hypothermia and fatal epileptic events, in a model of mitochondrial disease.

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