238 results found
    1. Structural Biology and Molecular Biophysics

    A Dynamic molecular basis for malfunction in disease mutants of p97/VCP

    Anne K Schuetz, Lewis E Kay
    Point mutations in a ubiquitous human ATPase called p97/VCP deregulate inter-domain communication, resulting in impaired binding of an adaptor that recruits p97 to endosomal pathways and leading to a degenerative disease of bone, muscle and neurons.
    1. Neuroscience

    Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants

    Ting Zhang et al.
    A potential medication has been found for the brain and muscle degenerative diseases caused by mutant forms of Valosin-Containing Protein.
    1. Cell Biology

    Mfn2 ubiquitination by PINK1/parkin gates the p97-dependent release of ER from mitochondria to drive mitophagy

    Gian-Luca McLelland et al.
    A crucial step during the mitophagy cascade involves the disassembly of connections between mitochondria and the endoplasmic reticulum via the retrotranslocation of Mfn2 tethering complexes by the Parkinson's disease genes PARKIN and PINK1, as well as the ATPase VCP/p97.
    1. Cell Biology

    p97-dependent retrotranslocation and proteolytic processing govern formation of active Nrf1 upon proteasome inhibition

    Senthil K Radhakrishnan et al.
    The enzyme p97/VCP regulates the activity of the transcription factor Nrf1 to promote increased transcription of genes that encode proteasome subunits following inhibition of the proteasome.
    1. Neuroscience

    Neurogliaform cortical interneurons derive from cells in the preoptic area

    Mathieu Niquille et al.
    In vivo genetic lineage-tracing reveals the developmental trajectory of neurogliaform cells, the main effectors of a powerful inhibitory motif in the neocortex.
    1. Neuroscience

    Plasticity of calcium-permeable AMPA glutamate receptors in Pro-opiomelanocortin neurons

    Shigetomo Suyama et al.
    Neurons in the hypothalamus that promote satiety manifest plasticity of their response to the excitatory transmitter, glutamate, by re-organizing the composition of a specific glutamare receptor.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    Tandem hnRNP A1 RNA recognition motifs act in concert to repress the splicing of survival motor neuron exon 7

    Irene Beusch et al.
    The structural model of hnRNP A1 shows that it can bind with both RRMs to RNA, which is shown to be relevant for the SMN2 exon 7 splicing mechanism in vivo.
    1. Cancer Biology

    TP53 drives invasion through expression of its Δ133p53β variant

    Gilles Gadea et al.
    Clinical analysis corroborated with experimental data demonstrate that Δ133p53β, a naturally occurring isoform of p53 protein, induces a pro-metastatic program and is a clinical indicator of metastasis, independently of TP53 mutation status.
    1. Cell Biology
    2. Neuroscience

    Presynaptic morphology and vesicular composition determine vesicle dynamics in mouse central synapses

    Laurent Guillaud et al.
    In central synapses, the mobility and supply of synaptic vesicles are determined by two independent biological factors: the morphological and structural organization of nerve terminals and the molecular signature of vesicles.
    1. Chromosomes and Gene Expression

    RNA Polymerase II cluster dynamics predict mRNA output in living cells

    Won-Ki Cho et al.
    Quantitative super resolution imaging, in live mammalian cells, reveals a direct relationship between protein clustering dynamics and the number of mRNA transcribed at an endogenous gene locus.

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