1,571 results found
    1. Medicine
    2. Neuroscience

    The mechanism underlying transient weakness in myotonia congenita

    Jessica H Myers, Kirsten Denman ... Mark M Rich
    Transient weakness in myotonia congenita is caused by depolarization secondary to activation of persistent Na+ current in skeletal muscle.
    1. Cell Biology
    2. Neuroscience

    The 133-kDa N-terminal domain enables myosin 15 to maintain mechanotransducing stereocilia and is essential for hearing

    Qing Fang, Artur A Indzhykulian ... Jonathan E Bird
    Two myosin 15 isoforms are required separately for the development and long-term maintenance of hearing.
    1. Medicine
    2. Immunology and Inflammation

    Chronic muscle weakness and mitochondrial dysfunction in the absence of sustained atrophy in a preclinical sepsis model

    Allison M Owen, Samir P Patel ... Hiroshi Saito
    Sepsis-induced long-term muscle weakness was reproduced using a refined murine model, which was accompanied by mitochondrial dysfunction in the absence of sustained atrophy, suggesting the promise of mitochondria-targeted post-sepsis therapies.
    1. Neuroscience

    Microsecond interaural time difference discrimination restored by cochlear implants after neonatal deafness

    Nicole Rosskothen-Kuhl, Alexa N Buck ... Jan WH Schnupp
    Early deaf human CI users are often insensitive to sub-millisecond interaural time differences (ITDs); however, with synchronized CIs, early deafened rats learned to lateralize small ITDs near 50 µs.
    1. Structural Biology and Molecular Biophysics

    Structural relationship between the putative hair cell mechanotransduction channel TMC1 and TMEM16 proteins

    Angela Ballesteros, Cristina Fenollar-Ferrer, Kenton Jon Swartz
    The structural relationship between TMC and TMEM16 proteins provides insight into the structure and functional mechanisms of the mechanotransduction channel complex in hair cells.
    1. Cell Biology
    2. Neuroscience

    PDZD7-MYO7A complex identified in enriched stereocilia membranes

    Clive P Morgan, Jocelyn F Krey ... Peter G Barr-Gillespie
    A new purification method for stereocilia membranes enables efficient immunoaffinity purification of rare protein complexes from hair cell stereocilia, including the newly described complex of deafness genes PDZD7 and MYO7A.
    1. Neuroscience

    Integration of Tmc1/2 into the mechanotransduction complex in zebrafish hair cells is regulated by Transmembrane O-methyltransferase (Tomt)

    Timothy Erickson, Clive P Morgan ... Teresa Nicolson
    A zebrafish model for a particular form of human deafness (DFNB63) changes our view of this disease by revealing a defect in the localization of Transmembrane channel-like proteins that are essential for mechanotransduction in sensory cells.
    1. Neuroscience

    Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function

    Bo Zhao, Zizhen Wu, Ulrich Müller
    Super-resolution microscopy reveals a highly organized compartment in the stereocilia of mechanosensory hair cells of the inner ear, which is critical for hair cell function and affected in disease.
    1. Cell Biology

    Impaired skeletal muscle mitochondrial pyruvate uptake rewires glucose metabolism to drive whole-body leanness

    Arpit Sharma, Lalita Oonthonpan ... Eric B Taylor
    Skeletal muscle mitochondrial pyruvate carrier disruption increases muscle fatty acid oxidation and systemic glucose turnover that drive whole-body leanness.
    1. Cell Biology

    Potassium dependent rescue of a myopathy with core-like structures in mouse

    M Gartz Hanson, Jonathan J Wilde ... Lee Niswander
    A mouse model of human muscle myopathy is used to provide mechanistic insight, identify possible biomarkers of disease, and suggest possible therapeutic strategies to alleviate muscle weakness.

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