66 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 et al.
    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. Cell Biology
    2. Neuroscience

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

    Pranesh Padmanabhan et al.
    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

    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. 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.
    Insight
    Available as:
    • HTML
    • PDF
    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. 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 et al.
    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.
    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. 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. Cell Biology
    2. Neuroscience

    Small-molecule G-quadruplex stabilizers reveal a novel pathway of autophagy regulation in neurons

    Jose F Moruno-Manchon et al.
    G-quadruplex DNA regulates autophagy in neurons.
    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.

Refine your results by:

Type
Research categories