54 results found
    1. Medicine
    2. Neuroscience

    PMCA-generated prions from the olfactory mucosa of patients with Fatal Familial Insomnia cause prion disease in mice

    Edoardo Bistaffa et al.
    The olfactory mucosa of patients with fatal familial insomnia contains traces of infectious prions, and their analyses could support the clinical diagnosis and elucidate disease-specific mechanisms.
    1. Cell Biology

    Spatial quality control bypasses cell-based limitations on proteostasis to promote prion curing

    Courtney L Klaips et al.
    Cell division imposes a limit on proteostasis capacity by reducing chaperone accumulation, but chaperone-substrate interactions reverse these events to allow clearance of even chronically misfolded protein amyloids.
    1. Microbiology and Infectious Disease

    The ER membrane protein complex is required to ensure correct topology and stable expression of flavivirus polyproteins

    Ashley M Ngo et al.
    The ER membrane protein complex (EMC) facilitates the correct topology of the flavivirus non-structural proteins NS4A and NS4B at the ER membrane critical for viral replication.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Guanidine hydrochloride reactivates an ancient septin hetero-oligomer assembly pathway in budding yeast

    Courtney R Johnson et al.
    A naturally-occurring small molecule acts as a chemical chaperone in vivo to alter the folding of a budding yeast septin and promote an oligomerization pathway that was lost during evolution.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Proteotoxicity from aberrant ribosome biogenesis compromises cell fitness

    Blake W Tye et al.
    Rapidly proliferating cells are at risk of compromised cell fitness due to proteostasis collapse from perturbations that interfere with ribosome biogenesis.
    1. Chromosomes and Gene Expression

    The genetic basis of aneuploidy tolerance in wild yeast

    James Hose et al.
    A single gene allele underlies differences in aneuploidy tolerance in yeast.
    1. Biochemistry and Chemical Biology
    2. Evolutionary Biology

    Enhanced ER proteostasis and temperature differentially impact the mutational tolerance of influenza hemagglutinin

    Angela M Phillips et al.
    Endoplasmic reticulum proteostasis factors enhance the mutational tolerance of influenza hemagglutinin, a model secretory pathway protein and therapeutic target, particularly improving the fitness of temperature-sensitive variants.
    1. Structural Biology and Molecular Biophysics
    2. Plant Biology

    Kinetics of the LOV domain of ZEITLUPE determine its circadian function in Arabidopsis

    Ashutosh Pudasaini et al.
    In the plant circadian clock photoreceptor ZEITLUPE, evolutionarily selected residues distinguish photocycle kinetics and allosteric signal transduction pathways to permit proper circadian timing.
    1. Cell Biology

    Compromising the 19S proteasome complex protects cells from reduced flux through the proteasome

    Peter Tsvetkov et al.
    Reducing the expression of 19S subunits shifts the ratio of 20S/26S proteasome complexes and provides a powerful survival advantage in the face of lethal proteasome inhibition.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation

    Lars Plate et al.
    Small molecule proteostasis regulators that activate the unfolded protein response transcription factor ATF6 reduce the secretion of amyloid disease-associated proteins.

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