12 results found
    1. Neuroscience

    Proteome profile of peripheral myelin in healthy mice and in a neuropathy model

    Sophie B Siems et al.
    A comprehensive compendium of myelin proteins in the peripheral nervous system has been created, alongside a method to address molecular diversity of myelin sheaths in health and disease.
    1. Cell Biology

    Molecular basis for dyneinopathies reveals insight into dynein regulation and dysfunction

    Matthew G Marzo et al.
    A mutagenesis screen in budding yeast sheds light on dynein regulation and function, and reveals the molecular basis for disease in patients suffering from neuropathies caused by dynein dysfunction.
    1. Neuroscience

    The critical role of membralin in postnatal motor neuron survival and disease

    Bo Yang et al.
    Membralin mutant mice manifest a severe and early-onset motor neuron disease with defects in endoplasmic reticulum-associated protein degradation.
    1. Neuroscience

    Charcot-Marie-Tooth 2B mutations in rab7 cause dosage-dependent neurodegeneration due to partial loss of function

    Smita Cherry et al.
    Mutations that reduce the functioning of rab7 may underlie the degeneration of sensory and motor nerves in Charcot-Marie-Tooth 2B disease, which suggests increasing endolysosomal function as a therapy.
    1. Neuroscience

    Neuronal complexity is attenuated in preclinical models of migraine and restored by HDAC6 inhibition

    Zachariah Bertels et al.
    In models of chronic migraine, neuronal complexity is diminished in head-pain processing regions but restored through HDAC6 inhibition, which increases tubulin acetylation and cytoskeletal flexibility, and CGRP receptor blockade.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    The Ormdl genes regulate the sphingolipid synthesis pathway to ensure proper myelination and neurologic function in mice

    Benjamin A Clarke et al.
    The ORMDL proteins function to restrain the de novo sphingolipid biosynthetic pathway during myelination, when there is a high demand for sphingolipids to prevent excessive accumulation of metabolic intermediates.
    1. Cell Biology
    2. Neuroscience

    Burst mitofusin activation reverses neuromuscular dysfunction in murine CMT2A

    Antonietta Franco et al.
    A trial of mitofusin activation shows neuron regeneration and phenotype reversal in vitro and in vivo in experimental Charcot–Marie–Tooth disease type 2A caused by mitofusin 2 mutations.
    1. Neuroscience

    Egr2 induction in spiny projection neurons of the ventrolateral striatum contributes to cocaine place preference in mice

    Diptendu Mukherjee et al.
    The ventrolateral striatum is found to be recruited during the encoding of cocaine preference, within which Egr2-expressing ensembles are functionally important, as is the expression of Egr2 within them.
    1. Neuroscience

    Schwann cells, but not Oligodendrocytes, Depend Strictly on Dynamin 2 Function

    Daniel Gerber et al.
    In the peripheral nervous system, the large GTPase dynamin 2 is required for Schwann cell survival, developmental radial sorting of axons, myelination, and myelin maintenance.
    1. Cell Biology

    Site-specific glycosylation regulates the form and function of the intermediate filament cytoskeleton

    Heather J Tarbet et al.
    The in vivo modification of the canonical intermediate filament protein vimentin with O-linked beta-N-acetylglucosamine affects its function in filament assembly, cell migration and host-pathogen interactions.

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