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The presence of giant granules in peripheral blood leukocytes is an important hallmark of CHS. Here we prepared induced pluripotent stem cells (iPSCs) from CHS patients (CHS\u2010iPSCs) and differentiated them into hematopoietic cells to model the disease phenotypes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Fibroblasts were obtained from two CHS patients and then reprogrammed into iPSCs. The iPSCs were differentiated into myeloid cells; the size of the cytosolic granules was quantified by May\u2010Grunwald Giemsa staining and myeloperoxidase staining.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Two clones of iPSCs were established from each patient. The differentiation efficiency to CD33<jats:sup>+<\/jats:sup>CD45<jats:sup>+<\/jats:sup> myeloid cells was not significantly different in CHS\u2010iPSCs compared with control iPSCs, but significantly larger granules were observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We succeeded in reproducing a characteristic cellular phenotype, giant granules in myeloid cells, using CHS\u2010iPSCs, demonstrating that iPSCs can be used to model the pathogenesis of CHS patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ped.15390","type":"journal-article","created":{"date-parts":[[2022,10,19]],"date-time":"2022-10-19T07:08:11Z","timestamp":1666163291000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["<scp>iPS<\/scp> cells from <scp>Chediak\u2010Higashi<\/scp> syndrome patients recapitulate the giant granules in myeloid cells"],"prefix":"10.1111","volume":"64","author":[{"given":"Shigeharu","family":"Oh","sequence":"first","affiliation":[{"name":"Department of Clinical Application, Center for iPS Cell Research and Application Kyoto University  Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6412-2686","authenticated-orcid":false,"given":"Akira","family":"Niwa","sequence":"additional","affiliation":[{"name":"Department of Clinical Application, Center for iPS Cell Research and Application Kyoto University  Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayako","family":"Nagahashi","sequence":"additional","affiliation":[{"name":"Department of Fundamental Cell Technologies, Center for iPS Cell Research and Application Kyoto University  Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isao","family":"Asaka","sequence":"additional","affiliation":[{"name":"Department of Fundamental Cell Technologies, Center for iPS Cell Research and Application Kyoto University  Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsutoshi","family":"Nakahata","sequence":"additional","affiliation":[{"name":"Department of Clinical Application, Center for iPS Cell Research and Application Kyoto University  Kyoto Japan"},{"name":"Department of Fundamental Cell Technologies, Center for iPS Cell Research and Application Kyoto University  Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8813-3614","authenticated-orcid":false,"given":"Megumu K.","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Clinical Application, Center for iPS Cell Research and Application Kyoto University  Kyoto 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