10,271 results found
    1. Cell Biology
    2. Neuroscience

    A novel ALS-associated variant in UBQLN4 regulates motor axon morphogenesis

    Brittany M Edens et al.
    A novel ALS-associated variant in UBQLN4 impairs proteasome function and beta-catenin degradation to drive aberrant axon morphogenesis in motor neurons.
    1. Neuroscience
    2. Stem Cells and Regenerative Medicine

    Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neurons

    Disi An et al.
    Stem cell-derived motor neurons with differential ALS vulnerability identified proteasome activity as a possible mechanism that explains their differential sensitivity.
    1. Cell Biology

    Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology

    Peipei Zhang et al.
    OptoGranules reveal the function of G3BP1 as a stress granule scaffold and demonstrate that protracted stress granule assembly is sufficient to drive neurodegeneration and the evolution of ALS-FTD pathology.
    1. Computational and Systems Biology

    Metage2Metabo, microbiota-scale metabolic complementarity for the identification of key species

    Arnaud Belcour et al.
    Computational models and software connect metagenomics to metabolic network reconstruction, assess metabolic complementarity between species, and identify critical species associated to functions of interest.
    1. Neuroscience

    Dynamic neuromuscular remodeling precedes motor-unit loss in a mouse model of ALS

    Éric Martineau et al.
    Motor axons undergo dynamic branch-specific changes for weeks before complete neuronal degeneration in a model of amyotrophic lateral sclerosis, highlighting the importance of peripheral factors, intrinsic and extrinsic to motoneurons.
    1. Evolutionary Biology

    Splicing repression allows the gradual emergence of new Alu-exons in primate evolution

    Jan Attig et al.
    During evolutionary progression of Alu-exons, repressive U-tracts buffer sudden gains in 3' splice site strength and prevent inclusion of cryptic exons.
    1. Cell Biology

    Transcriptional activator TAp63 is upregulated in muscular atrophy during ALS and induces the pro-atrophic ubiquitin ligase Trim63

    Yannick von Grabowiecki et al.
    Atrophic muscles of patients and animal models developing amyotrophic lateral sclerosis show an upregulation of TAp63 that stimulates the expression of a pro-atrophic ubiquitin ligase.
    1. Cell Biology
    2. Structural Biology and Molecular Biophysics

    Systematic studies of all PIH proteins in zebrafish reveal their distinct roles in axonemal dynein assembly

    Hiroshi Yamaguchi et al.
    Zebrafish genetics and cryo-electron tomography reveal distinct roles of all vertebrate PIH family proteins in axonemal dynein assembly and cilia/flagella motions, assigning specific dynein subtypes to each PIH protein.
    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 et al.
    Idiopathic patients along a complex motor neuron disease-dementia spectrum exhibit mRNA splicing changes that are due to multi-protein insolubility.
    1. Evolutionary Biology
    2. Genetics and Genomics

    Demographic reconstruction from ancient DNA supports rapid extinction of the great auk

    Jessica E Thomas et al.
    Reconstruction of great auk population dynamics suggests that hunting pressure alone could have been responsible for their extinction, demonstrating that even abundant, widespread species can be vulnerable to intense exploitation.

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