1,417 results found
    1. Immunology and Inflammation
    2. Microbiology and Infectious Disease

    IRF4 haploinsufficiency in a family with Whipple’s disease

    Antoine Guérin et al.
    Autosomal dominant IRF4 deficiency is the first genetic etiology of Whipple's disease, a very rare chronic condition following a rather common infection by Tropheryma whipplei.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    An unfolded protein-induced conformational switch activates mammalian IRE1

    G Elif Karagöz et al.
    ER-stress sensing mechanism of the unfolded protein response sensor/transducer IRE1 is conserved from yeast to mammals, where in mammals, unfolded protein binding to IRE1's ER lumenal domain is coupled to its oligomerization and activation through an allosteric conformational change.
    1. Biochemistry and Chemical Biology

    The aspartyl protease DDI2 activates Nrf1 to compensate for proteasome dysfunction

    Shun Koizumi et al.
    Peptidase activity of DDI2 is required to activate Nrf1 in order to enable proteasome recovery in response to proteasome inhibition.
    1. Cell Biology

    The unfolded protein response and endoplasmic reticulum protein targeting machineries converge on the stress sensor IRE1

    Diego Acosta-Alvear et al.
    The coordination of protein targeting to the endoplasmic reticulum and the unfolded protein response through the stress sensor IRE1 ensures quality control of the secreted and transmembrane proteomes.
    1. Chromosomes and Gene Expression
    2. Stem Cells and Regenerative Medicine

    TERRA regulate the transcriptional landscape of pluripotent cells through TRF1-dependent recruitment of PRC2

    Rosa María Marión et al.
    Telomeric TRF1 controls the transcriptional programmes of pluripotent stem cells by recruiting PRC2 to pluripotency and differentiation genes by controlling the expression of those gene sites and the binding of TERRA RNAs to them.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    In vitro FRET analysis of IRE1 and BiP association and dissociation upon endoplasmic reticulum stress

    Megan C Kopp et al.
    Quantitative FRET UPR induction assay is used to measure IRE1 and BIP association and dissociation by a variety of ER misfolded proteins and by an important BiP substrate-binding domain mutant, significantly enhancing the evidence for the allosteric UPR induction model.
    1. Chromosomes and Gene Expression
    2. Human Biology and Medicine

    Bromodomain inhibition of the transcriptional coactivators CBP/EP300 as a therapeutic strategy to target the IRF4 network in multiple myeloma

    Andrew R Conery et al.
    CBP/EP300 bromodomain inhibitors have a therapeutic application in oncology via targeting the IRF4 transcriptional program, a clinically validated network critical for multiple myeloma cell viability.
    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. Cell Biology
    2. Developmental Biology

    Proteasome dysfunction triggers activation of SKN-1A/Nrf1 by the aspartic protease DDI-1

    Nicolas J Lehrbach, Gary Ruvkun
    Post-translational processing of the SKN-1A/Nrf1 transcription factor regulates proteasome expression.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    Noncanonical binding of BiP ATPase domain to Ire1 and Perk is dissociated by unfolded protein CH1 to initiate ER stress signaling

    Marta Carrara et al.
    The chaperone protein BiP forms complexes with Ire1 and Perk that dissociate when unfolded proteins bind to BiP to activate the unfolded protein response in the ER.

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