31 results found
    1. Cell Biology
    2. Neuroscience

    C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import

    Lindsey R Hayes et al.
    Poly-PR and poly-GR interact with importin β, disrupt importin-cargo loading, and inhibit nuclear import in permeabilized cells in a manner that can be rescued by RNA.
    1. Cell Biology
    2. Neuroscience

    Implementation of an antibody characterization procedure and application to the major ALS/FTD disease gene C9ORF72

    Carl Laflamme et al.
    An easy-to-implement antibody validation pipeline addresses the reproducibility crisis resulting from the use of non-specific antibodies.
    1. Biochemistry and Chemical Biology

    The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains

    Erin G Conlon et al.
    The C9orf72 expansion binds and sequesters the splicing factor hnRNP H leading to insoluble G-quadruplex aggregates that functionally reduce hnRNP H, thereby producing splicing defects.
    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. Neuroscience

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

    Barbara Celona et al.
    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. Biochemistry and Chemical Biology

    RNA-Binding Proteins: A matter of balance

    Aaron D Gitler, John D Fryer
    New analyses shift the view that some forms of amyotrophic lateral sclerosis and frontotemporal dementia are due to defects in a single RNA-binding protein.
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    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Multiplex image-based autophagy RNAi screening identifies SMCR8 as ULK1 kinase activity and gene expression regulator

    Jennifer Jung et al.
    An image-based multiplex autophagosome RNAi screen targeting all Rab GTPases as well as their GAPs and GEFs identifies the Rab GEF SMCR8 as multifaceted autophagy modulator, which regulates kinase activity and gene expression of ULK1.
    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. Biochemistry and Chemical Biology

    Serine is the major residue for ADP-ribosylation upon DNA damage

    Luca Palazzo et al.
    Serine residues in proteins are the primary targets for PARP-dependent ADP-ribosylation signalling upon DNA damage.
    1. Developmental Biology
    2. Genetics and Genomics

    Zygotic gene activation in the chicken occurs in two waves, the first involving only maternally derived genes

    Young Sun Hwang et al.
    In Aves, the first wave of transcription is derived exclusively from the maternal genome.

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