{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T01:03:08Z","timestamp":1792371788134,"version":"4.4.0"},"reference-count":50,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2022,7,11]],"date-time":"2022-07-11T00:00:00Z","timestamp":1657497600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Laboratory of Excellence GR-Ex","award":["ANR-11-LABX-0051"],"award-info":[{"award-number":["ANR-11-LABX-0051"]}]},{"name":"labex GR-Ex fellowship and\/or scholarship","award":["ANR-11-LABX-0051"],"award-info":[{"award-number":["ANR-11-LABX-0051"]}]},{"name":"Soci\u00e9t\u00e9 Fran\u00e7aise d\u2019H\u00e9matologie","award":["ANR-11-LABX-0051"],"award-info":[{"award-number":["ANR-11-LABX-0051"]}]},{"name":"Universit\u00e9 Paris Cit\u00e9","award":["ANR-11-LABX-0051"],"award-info":[{"award-number":["ANR-11-LABX-0051"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Gaucher disease (GD) is caused by glucocerebrosidase deficiency leading to the accumulation of sphingolipids in macrophages named \u201cGaucher\u2019s Cells\u201d. These cells are characterized by deregulated expression of cell surface markers, abnormal secretion of inflammatory cytokines, and iron sequestration. These cells are known to infiltrate tissues resulting in hematological manifestations, splenomegaly, and bone diseases. We have already demonstrated that Gaucher red blood cells exhibit altered properties suggesting their key role in GD clinical manifestations. We hypothesized that Gaucher\u2019s erythrocytes could be prone to premature destruction by macrophages contributing to the formation of altered macrophages and Gaucher-like cells. We conducted in vitro experiments of erythrophagocytosis using erythrocytes from Gaucher\u2019s patients or healthy donors. Our results showed an enhanced erythrophagocytosis of Gaucher red blood cells compared to healthy red blood cells, which is related to erythrocyte sphingolipids overload and reduced deformability. Importantly, we showed elevated expression of the antigen-presenting molecules CD1d and MHC-II and of the iron-regulator hepcidin in macrophages, as well as enhanced secretion of the pro-inflammatory cytokine IL-1\u03b2 after phagocytosis of GD erythrocytes. These results strongly suggested that erythrophagocytosis in GD contribute to phenotypic modifications in macrophages. This present study shows that erythrocytes-macrophages interactions may be crucial in GD pathophysiology and pathogenesis.<\/jats:p>","DOI":"10.3390\/ijms23147640","type":"journal-article","created":{"date-parts":[[2022,7,11]],"date-time":"2022-07-11T21:57:53Z","timestamp":1657576673000},"page":"7640","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Phagocytosis of Erythrocytes from Gaucher Patients Induces Phenotypic Modifications in Macrophages, Driving Them toward Gaucher Cells"],"prefix":"10.3390","volume":"23","author":[{"given":"Lucie","family":"Dupuis","sequence":"first","affiliation":[{"name":"INSERM, UMR_S1134, BIGR, Universit\u00e9 Paris Cit\u00e9 and Universit\u00e9 des Antilles, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaux","family":"Chauvet","sequence":"additional","affiliation":[{"name":"INSERM, UMR_S1134, BIGR, Universit\u00e9 Paris Cit\u00e9 and Universit\u00e9 des Antilles, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Bourdelier","sequence":"additional","affiliation":[{"name":"INSERM, UMR_S1134, BIGR, Universit\u00e9 Paris Cit\u00e9 and Universit\u00e9 des Antilles, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micha\u00ebl","family":"Dussiot","sequence":"additional","affiliation":[{"name":"UMR_S1163, Sorbonne Paris Cit\u00e9, Institut Imagine, Laboratoire d\u2019Excellence GR-Ex, Universit\u00e9 Paris Cit\u00e9, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Belmatoug","sequence":"additional","affiliation":[{"name":"APHP CRML Maladies Lysosomales, Service de M\u00e9decine Interne, H\u00f4pital Beaujon, Sorbonne Universit\u00e9, F-92110 Clichy, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3251-1310","authenticated-orcid":false,"given":"Caroline","family":"Le Van Kim","sequence":"additional","affiliation":[{"name":"INSERM, UMR_S1134, BIGR, Universit\u00e9 Paris Cit\u00e9 and Universit\u00e9 des Antilles, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6551-4382","authenticated-orcid":false,"given":"Arnaud","family":"Ch\u00eane","sequence":"additional","affiliation":[{"name":"INSERM, UMR_S1134, BIGR, Universit\u00e9 Paris Cit\u00e9 and Universit\u00e9 des Antilles, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6519-4131","authenticated-orcid":false,"given":"M\u00e9lanie","family":"Franco","sequence":"additional","affiliation":[{"name":"INSERM, UMR_S1134, BIGR, Universit\u00e9 Paris Cit\u00e9 and Universit\u00e9 des Antilles, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1016\/S0950-3536(97)80033-9","article-title":"Gaucher\u2019s disease: Clinical features and natural history","volume":"10","author":"Cox","year":"1997","journal-title":"Baillieres Clin. 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