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    1. Neuroscience

    A size principle for recruitment of Drosophila leg motor neurons

    Anthony W Azevedo, Evyn S Dickinson ... John C Tuthill
    Motor neurons controlling the leg of the fruit fly exhibit a gradient of physiological and functional properties that correlate with the order in which they fire during behavior.
    1. Neuroscience

    Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106

    Barbara Celona, John von Dollen ... Brian L Black
    Zfp106 functions as an RNA binding protein, binds directly to GGGGCC RNA repeats, is required in motor neurons to prevent ALS-like neurodegeneration in mice, and can suppress neurotoxicity in an established fly model of ALS.
    1. Neuroscience

    Cerebellar nuclei cells produce distinct pathogenic spike signatures in mouse models of ataxia, dystonia, and tremor

    Meike E van der Heijden, Amanda M Brown ... Roy V Sillitoe
    Ataxia, dystonia, and tremor phenotypes have distinct and generalizable cerebellar interposed nucleus spike signatures in mice, with defining features identified using a classifier model and pathogenicity tested using optogenetic manipulations.
    1. Developmental Biology

    Multifaceted roles of microRNAs: From motor neuron generation in embryos to degeneration in spinal muscular atrophy

    Tai-Heng Chen, Jun-An Chen
    Summary review of microRNA roles during motor neuron generation and degeneration.
    1. Cell Biology
    2. Neuroscience

    Specific depletion of the motor protein KIF5B leads to deficits in dendritic transport, synaptic plasticity and memory

    Junjun Zhao, Albert Hiu Ka Fok ... Kwok-On Lai
    Two homologous kinesin motors, KIF5A and KIF5B, have distinct functions in dendritic spine morphogenesis, and KIF5B plays a role in synaptic plasticity and memory.
    1. Biochemistry and Chemical Biology
    2. Medicine

    Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism

    Erin G Conlon, Delphine Fagegaltier ... James L Manley
    Idiopathic patients along a complex motor neuron disease-dementia spectrum exhibit mRNA splicing changes that are due to multi-protein insolubility.
    1. Developmental Biology
    2. Stem Cells and Regenerative Medicine

    A 3D culture model of innervated human skeletal muscle enables studies of the adult neuromuscular junction

    Mohsen Afshar Bakooshli, Ethan S Lippmann ... Penney M Gilbert
    Human skeletal muscle progenitors and motor neurons self-organize in three-dimensional co-culture to form functional neuromuscular junctions that developmentally mature from the embryonic to the adult state.
    1. Stem Cells and Regenerative Medicine

    An optogenetic cell therapy to restore control of target muscles in an aggressive mouse model of amyotrophic lateral sclerosis

    J Barney Bryson, Alexandra Kourgiantaki ... Linda Greensmith
    Stem-cell-based neural replacement, in combination with optogenetic stimulation, could represent a translationally viable therapeutic strategy to overcome atrophy and paralysis of targeted muscles in people living with amyotrophic lateral sclerosis.
    1. Neuroscience

    Neuromuscular Disease: Protecting the nerve terminals

    Jonathan D Glass
    Maintaining the connections between nerve cells and muscle could help to slow the progression of amyotrophic lateral sclerosis.
    Version of Record
    Insight
    1. Neuroscience

    Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS

    María de Lourdes Martínez-Silva, Rebecca D Imhoff-Manuel ... Marin Manuel
    The most vulnerable motor units lose a fundamental firing property before the denervation of their muscle fibers in ALS mice, changing our view of the role of excitability in neurodegeneration.