117 results found
    1. Biochemistry and Chemical Biology
    2. Neuroscience

    Targeted degradation of aberrant tau in frontotemporal dementia patient-derived neuronal cell models

    M Catarina Silva, Fleur M Ferguson ... Stephen J Haggarty
    A new Cereblon-recruiting bifunctional tau ligand, QC-01-175, promotes aberrant tau degradation and rescue of stress vulnerability in human neuronal cell models of tauopathy.
    1. Medicine
    2. Neuroscience

    Resting-state alterations in behavioral variant frontotemporal dementia are related to the distribution of monoamine and GABA neurotransmitter systems

    Lisa Hahn, Simon B Eickhoff ... Matthias L Schroeter
    Local brain functional activity reductions in bvFTD follow the distribution of serotonergic, GABAergic, and norepinephrinergic neurotransmitter systems, indicating a selective vulnerability and providing novel insights into the underlying disease mechanisms.
    1. Cell Biology
    2. Neuroscience

    Frontotemporal dementia mutant Tau promotes aberrant Fyn nanoclustering in hippocampal dendritic spines

    Pranesh Padmanabhan, Ramón Martínez-Mármol ... Frédéric A Meunier
    Super-resolution imaging reveals that the microtubule-associated protein Tau regulates the Fyn kinase organisation in dendrites, and that the frontotemporal dementia mutant Tau promotes aberrant Fyn clustering, potentially leading to synaptic dysfunction.
    1. Neuroscience

    Distinctive whole-brain cell types predict tissue damage patterns in thirteen neurodegenerative conditions

    Veronika Pak, Quadri Adewale ... Yasser Iturria-Medina
    Major cell types across various brain regions distinctively associate with tissue loss in 13 neurodegenerative conditions.
    1. Neuroscience

    Model-based whole-brain perturbational landscape of neurodegenerative diseases

    Yonatan Sanz Perl, Sol Fittipaldi ... Enzo Tagliazucchi
    The combination of deep learning with whole-brain computational models reveals the low-dimensional representation of neurodegenerative diseases, which emerges from a highly multidimensional brain, providing valuable insight into pathological states' diagnostic, prognosis, and treatment response.
    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.
    Version of Record
    Insight
    1. Neuroscience

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

    Ting Zhang, Prashant Mishra ... Ming Guo
    A potential medication has been found for the brain and muscle degenerative diseases caused by mutant forms of Valosin-Containing Protein.
    1. Neuroscience

    Impact of long- and short-range fibre depletion on the cognitive deficits of fronto-temporal dementia

    Melissa Savard, Tharick A Pascoal ... Pedro Rosa-Neto
    While semantic symptoms in fronto-temporal dementia patients were mainly dependent on short-range white-matter fibre disruption, long-range white-matter fibres damage was the major contributor to executive dysfunction, highlighting the importance of controlling for risk factors associated with deep white-matter disease.
    1. Biochemistry and Chemical Biology
    2. Genetics and Genomics

    SERBP1 interacts with PARP1 and is present in PARylation-dependent protein complexes regulating splicing, cell division, and ribosome biogenesis

    Kira Breunig, Xuifen Lei ... Luiz O Penalva
    SERBP1 is associate with protein complexes regulating splicing, cell division, and ribosomal biogenesis that likely depend on G quadruplex (G4) binding and polyADP-ribosylation (PARylation) for their assembly and function.
    1. Biochemistry and Chemical Biology
    2. Neuroscience

    Multiple pathways of toxicity induced by C9orf72 dipeptide repeat aggregates and G4C2 RNA in a cellular model

    Frédéric Frottin, Manuela Pérez-Berlanga ... Mark S Hipp
    Protein aggregates resulting from mutations in C9orf72 impair different aspects of cellular quality control in the cytosol and the nucleus, but mRNA-mediated effects contribute more strongly to toxicity.

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