{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T05:52:56Z","timestamp":1784872376005,"version":"3.55.0"},"reference-count":52,"publisher":"National Academy of Sciences","issue":"46","license":[{"start":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T00:00:00Z","timestamp":1699315200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"HHS | National Institutes of Health","doi-asserted-by":"publisher","award":["AI125581"],"award-info":[{"award-number":["AI125581"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2023,11,14]]},"abstract":"<jats:p>\n                    In a forward genetic screen of mice with\n                    <jats:italic>N<\/jats:italic>\n                    -ethyl-\n                    <jats:italic>N<\/jats:italic>\n                    -nitrosourea-induced mutations for aberrant immune function, we identified animals with low percentages of B220\n                    <jats:sup>+<\/jats:sup>\n                    cells in the peripheral blood. The causative mutation was in\n                    <jats:italic>Ier3ip1<\/jats:italic>\n                    , encoding immediate early response 3 interacting protein 1 (IER3IP1), an endoplasmic reticulum membrane protein mutated in an autosomal recessive neurodevelopmental disorder termed Microcephaly with simplified gyration, Epilepsy and permanent neonatal Diabetes Syndrome (MEDS) in humans. However, no immune function for IER3IP1 had previously been reported. The viable hypomorphic\n                    <jats:italic>Ier3ip1<\/jats:italic>\n                    allele uncovered in this study, identical to a reported\n                    <jats:italic>IER3IP1<\/jats:italic>\n                    variant in a MEDS patient, reveals an essential hematopoietic-intrinsic role for IER3IP1 in B cell development and function. We show that IER3IP1 forms a complex with the Golgi transmembrane protein 167A and limits activation of the unfolded protein response mediated by inositol-requiring enzyme-1\u03b1 and X-box binding protein 1 in B cells. Our findings suggest that B cell deficiency may be a feature of MEDS.\n                  <\/jats:p>","DOI":"10.1073\/pnas.2312810120","type":"journal-article","created":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T13:59:04Z","timestamp":1699365544000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":12,"title":["Essential requirement for IER3IP1 in B cell development"],"prefix":"10.1073","volume":"120","author":[{"given":"Xue","family":"Zhong","sequence":"first","affiliation":[{"name":"Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1178-1642","authenticated-orcid":false,"given":"James J.","family":"Moresco","sequence":"additional","affiliation":[{"name":"Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie","family":"Keller","sequence":"additional","affiliation":[{"name":"Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle Renee","family":"Lazaro","sequence":"additional","affiliation":[{"name":"Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Ely","sequence":"additional","affiliation":[{"name":"Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2049-7291","authenticated-orcid":false,"given":"Eva Marie Y.","family":"Moresco","sequence":"additional","affiliation":[{"name":"Center 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