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Characteristic of the disease is the accumulation of \u03b1\u2010Gal\u2010A substrates, primarily the glycosphingolipids (GSLs) globotriaosylceramide and globotriaosylsphingosine. Thrombotic events are a significant concern for Fabry patients, with strokes contributing to a significant decrease in overall lifespan. Currently, the mechanisms underlying the increased risk of thrombotic events experienced by Fabry patients are incompletely defined. Using a rat model of Fabry disease, we provide an improved understanding of the mechanisms linking GSL accumulation to thrombotic risk. We found that \u237a\u2010Gal A\u2010deficient rats accumulate myeloid\u2010derived leukocytes at sites of GSL accumulation, including in the bone marrow and circulation, and that myeloid\u2010derived leukocyte and megakaryocyte populations were prominent among cell types that accumulated GSLs. In the circulation, \u237a\u2010Gal A\u2010deficient rats had increases in cytokine\u2010producing cell types and a corresponding elevation of pro\u2010inflammatory cytokines. Lastly, circulating platelets from \u237a\u2010Gal A\u2010deficient rats accumulated a similar set of \u237a\u2010Galactosidase\u2010A substrates as was observed in megakaryocytes in the bone marrow, and exhibited increased platelet binding to fibrinogen in microfluidic and flow cytometric assays.<\/jats:p>","DOI":"10.1096\/fj.202001727rr","type":"journal-article","created":{"date-parts":[[2021,7,28]],"date-time":"2021-07-28T13:14:25Z","timestamp":1627478065000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Platelet and myeloid cell phenotypes in a rat model of Fabry disease"],"prefix":"10.1096","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6077-0475","authenticated-orcid":false,"given":"Adam J.","family":"Kanack","sequence":"first","affiliation":[{"name":"Department of Biochemistry Medical College of Wisconsin Milwaukee WI 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