717 results found
    1. Biochemistry and Chemical Biology

    Determining composition of micron-scale protein deposits in neurodegenerative disease by spatially targeted optical microproteomics

    Kevin C Hadley et al.
    STOMP is a technique that can determine the proteomic composition of any feature that is identifiable by laser scanning microscopy and is at least one cubic micron in size.
    1. Neuroscience

    Enhanced insulin signalling ameliorates C9orf72 hexanucleotide repeat expansion toxicity in Drosophila

    Magda L Atilano et al.
    Modulation of insulin signalling could be an effective therapeutic approach against hexanucleotide repeat expansion related to c9ALS/FTD neurodegenerative diseases.
    1. Computational and Systems Biology
    2. Neuroscience

    Differences in topological progression profile among neurodegenerative diseases from imaging data

    Sara Garbarino et al.
    Computational-driven, imaging-based topological profiles of neurodegeneration differ substantially in different neurodegenerative conditions, suggesting distinct modes of dependence of the pathological spread on the underlying connectivity.
    1. Structural Biology and Molecular Biophysics
    2. Neuroscience

    Neurodegenerative disease mutations in TREM2 reveal a functional surface and distinct loss-of-function mechanisms

    Daniel L Kober et al.
    Structural, biophysical, and functional analyses reveal that mutations in TREM2 trigger either misfolding or reduced binding to cell-surface glycosaminoglycans, which segregate with neurodegenerative disease link and highlight a functional surface linked to the pathogenesis of Alzheimer's disease.
    1. Neuroscience

    A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity

    Yuwei Yao et al.
    A cell-surface receptor called Gpr52 is able to lower the levels of the disease-causing protein mutant huntingtin and suppress its toxicity when knocked-down, making this receptor a promising drug target in Huntington's disease.
    1. Neuroscience

    Increased expression of heme-binding protein 1 early in Alzheimer's disease is linked to neurotoxicity

    Oleksandr Yagensky et al.
    Increased levels of brain Hebp1 starting from the presymptomatic stage of Alzheimer’s disease contributes to progressive neuronal loss by triggering mitochondrial-dependent apoptosis in neurons exposed to elevated heme.
    1. Neuroscience
    2. Structural Biology and Molecular Biophysics

    Two new polymorphic structures of human full-length alpha-synuclein fibrils solved by cryo-electron microscopy

    Ricardo Guerrero-Ferreira et al.
    Two new polymorphic structures of recombinant human alpha-synuclein fibrils show striking differences to previous structures, while familial PD mutation sites remain crucial for protofilament interaction and fibril stability.
    1. Microbiology and Infectious Disease
    2. Neuroscience

    The sheddase ADAM10 is a potent modulator of prion disease

    Hermann C Altmeppen et al.
    A lack of ADAM10-mediated shedding increases prion protein levels at the plasma membrane and promotes the generation of pathological prion proteins, which accelerates prion disease in mice.
    1. Neuroscience

    TRIM28 regulates the nuclear accumulation and toxicity of both alpha-synuclein and tau

    Maxime WC Rousseaux et al.
    Convergent screens targeting the levels of alpha-synuclein and tau identify TRIM28 as a driver of their stability, nuclear accumulation and subsequent toxicity.
    1. Cell Biology
    2. Structural Biology and Molecular Biophysics

    Intrinsically aggregation-prone proteins form amyloid-like aggregates and contribute to tissue aging in Caenorhabditis elegans

    Chaolie Huang et al.
    Age-dependent protein aggregation closely resembles protein aggregation associated with neurodegenerative diseases and other amyloidoses, and initiates early functional decline in different tissues.

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