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Here we show in a child with microthrombocytopenia, eosinophilia and inflammatory disease, a homozygous frameshift mutation in\n                    <jats:italic>ARPC1B<\/jats:italic>\n                    (p.Val91Trpfs*30). Platelet lysates reveal no ARPC1B protein and greatly reduced Arp2\/3 complex. Missense\n                    <jats:italic>ARPC1B<\/jats:italic>\n                    mutations are identified in an unrelated patient with similar symptoms and ARPC1B deficiency. ARPC1B-deficient platelets are microthrombocytes similar to those seen in Wiskott\u2013Aldrich syndrome that show aberrant spreading consistent with loss of Arp2\/3 function. Knockout of\n                    <jats:italic>ARPC1B<\/jats:italic>\n                    in megakaryocytic cells results in decreased proplatelet formation, and as observed in platelets from patients, increased ARPC1A expression. Thus loss of ARPC1B produces a unique set of platelet abnormalities, and is associated with haematopoietic\/immune symptoms affecting cell lineages where this isoform predominates. In agreement with recent experimental studies, our findings suggest that ARPC1 isoforms are not functionally interchangeable.\n                  <\/jats:p>","DOI":"10.1038\/ncomms14816","type":"journal-article","created":{"date-parts":[[2017,4,3]],"date-time":"2017-04-03T05:32:38Z","timestamp":1491197558000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":221,"title":["Loss of the Arp2\/3 complex component ARPC1B causes platelet abnormalities and predisposes to inflammatory disease"],"prefix":"10.1038","volume":"8","author":[{"given":"Walter H. 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