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Access type: UnknownAuthor: search.filters.author.Andrew J. Oler

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    HEM1 deficiency disrupts mTORC2 and F-actin control in inherited immunodysregulatory disease
    (2020)
    Sarah A. Cook
    ;
    William A. Comrie
    ;
    POLI HARLOWE, MARIA CECILIA BERTA  
    ;
    Morgan Similuk
    ;
    Andrew J. Oler
    <jats:title>An inherited disorder makes WAVEs</jats:title> <jats:p> The WAVE regulatory complex (WRC) is a multiunit complex that regulates actin cytoskeleton formation. Although other actin-regulatory proteins modulate human immune responses, the precise role for the WRC has not yet been established. Cook <jats:italic>et al.</jats:italic> studied five patients from four unrelated families who harbor missense variants of the gene encoding the WRC component HEM1. These patients presented with recurrent infections and poor antibody responses, along with enhanced allergic and autoimmune disorders. HEM1 was found to be required for the regulation of cortical actin and granule release in T cells and also interacted with a key metabolic signaling complex contributing to the disease phenotype. By linking these interactions to immune function, this work suggests potential targets for future immunotherapies. </jats:p> <jats:p> <jats:italic>Science</jats:italic> , this issue p. <jats:related-article xmlns:xlink="http://www.w3.org/1999/xlink" ext-link-type="doi" issue="6500" page="202" related-article-type="in-this-issue" vol="369" xlink:href="10.1126/science.aay5663">202</jats:related-article> </jats:p>
      10Scopus© Citations 94
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    Immunopathological signatures in multisystem inflammatory syndrome in children and pediatric COVID-19
    (2022)
    Keith Sacco
    ;
    Riccardo Castagnoli
    ;
    Svetlana Vakkilainen
    ;
    Can Liu
    ;
    Ottavia M. Delmonte
    Scopus© Citations 204  11
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    Multicenter analysis of neutrophil extracellular trap dysregulation in adult and pediatric COVID-19
    (2022)
    Carmelo Carmona-Rivera
    ;
    Yu Zhang
    ;
    Kerry Dobbs
    ;
    Tovah E. Markowitz
    ;
    Clifton L. Dalgard
      13Scopus© Citations 30