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Adv."],"published-print":{"date-parts":[[2026,7,31]]},"abstract":"<jats:p>\n                    Fanconi anemia (FA) is characterized by defective DNA repair and chronic p53 activation, predisposing to acute myeloid leukemia through persistent genomic instability. The molecular adaptations enabling cell survival under chronic stress remain poorly understood. This study investigates the role of ZMAT3, a p53 responsive RNA binding protein overexpressed in FA deficient cells, to elucidate its role in cellular adaptation. Using FA patient fibroblasts and\n                    <jats:italic toggle=\"yes\">Fancg<\/jats:italic>\n                    KO mouse models, we launched transcriptomic profiling, lipidomic, and functional analyses. RNA-seq and lipidomic analyses revealed unexpected alterations in sphingolipid metabolism upon ZMAT3 depletion, linked to ceramide accumulation and downregulation of ASAH1 (acid ceramidase). Functionally, ZMAT3 depletion increased DNA damage and enhanced ferroptosis susceptibility, an iron dependent cell death mechanism. Mechanistically, our results showed that ZMAT3 promotes ASAH1 expression, limits ceramide accumulation and protects cells from ferroptosis induced death which could participate to preleukemic clonal evolution and pointing to areas for further exploration in FA-associated malignancies.\n                  <\/jats:p>","DOI":"10.1126\/sciadv.aeb6444","type":"journal-article","created":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T17:58:34Z","timestamp":1785520714000},"update-policy":"https:\/\/doi.org\/10.34133\/aaas_crossmark","source":"Crossref","is-referenced-by-count":0,"title":["ZMAT3 alleviates cell death in Fanconi anemia via adaptation of sphingolipid metabolism"],"prefix":"10.1126","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4957-2814","authenticated-orcid":true,"given":"Maeva","family":"Loock","sequence":"first","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, CNRS UMR9019, F-94805, Villejuif, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-7178-6807","authenticated-orcid":true,"given":"Blandine","family":"Lucchini","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, INSERM U1360, F-94805, Villejuif, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Latour","sequence":"additional","affiliation":[{"name":"INSERM U1342, Institut de la Leuc\u00e9mie Paris Saint-Louis, Universit\u00e9 Paris-Cit\u00e9, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margot","family":"Tissandier","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, CNRS UMR9019, F-94805, Villejuif, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6335-4506","authenticated-orcid":true,"given":"Marie","family":"Lhomme","sequence":"additional","affiliation":[{"name":"IHU-ICAN, Foundation for Innovation in Cardiometabolism and Nutrition, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7761-4228","authenticated-orcid":true,"given":"Maharajah","family":"Ponnaiah","sequence":"additional","affiliation":[{"name":"IHU-ICAN, Foundation for Innovation in Cardiometabolism and Nutrition, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucie","family":"Hernandez","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, Institut de Recherche Saint-Louis (IRSL), INSERM UMR1342\/CNRS EMR8000, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loic","family":"Maillard","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, Institut de Recherche Saint-Louis (IRSL), INSERM UMR1342\/CNRS EMR8000, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean","family":"Soulier","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, Institut de Recherche Saint-Louis (IRSL), INSERM UMR1342\/CNRS EMR8000, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6943-8858","authenticated-orcid":true,"given":"Olivier","family":"Bluteau","sequence":"additional","affiliation":[{"name":"Service de Biochimie Endocrinienne et Oncologique, Assistance publique - H\u00f4pitaux de Paris Sorbonne Universit\u00e9 Piti\u00e9 Salp\u00eatri\u00e8re, Paris, France."},{"name":"Sorbonne Universit\u00e9, INSERM UMR_S1166, Unit\u00e9 de recherche sur les maladies cardiovasculaires et m\u00e9taboliques, ICAN, F-75013 Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0550-4921","authenticated-orcid":true,"given":"Camille","family":"Lobry","sequence":"additional","affiliation":[{"name":"INSERM U1342, Institut de la Leuc\u00e9mie Paris Saint-Louis, Universit\u00e9 Paris-Cit\u00e9, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1726-1472","authenticated-orcid":true,"given":"Marie-Laure","family":"Arcangeli","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, INSERM U1360, F-94805, Villejuif, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7611-9644","authenticated-orcid":true,"given":"Wilfried","family":"Le Goff","sequence":"additional","affiliation":[{"name":"IHU-ICAN, Foundation for Innovation in Cardiometabolism and Nutrition, Paris, France."},{"name":"Sorbonne Universit\u00e9, INSERM UMR_S1166, Unit\u00e9 de recherche sur les maladies cardiovasculaires et m\u00e9taboliques, ICAN, F-75013 Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6530-2363","authenticated-orcid":true,"given":"Dominique","family":"Bluteau","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, CNRS UMR9019, F-94805, Villejuif, France."},{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, INSERM U1360, F-94805, Villejuif, France."},{"name":"Ecole Pratique des Hautes Etudes, PSL University, Paris, France."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1201454"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(02)03004-1"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1204722"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M203594200"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41388-023-02601-0"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1038\/emboj.2012.286"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/acel.13557"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2020.10.022"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41591-018-0043-5"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-024-83369-5"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr583"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/cddis.2012.33"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/path.4321"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0169-328X(99)00146-1"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2014.08.001"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrm.2016.48"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.stem.2012.05.013"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature11368"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature25154"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V91.11.4092"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI31772"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2008.2129"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.stem.2016.03.002"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.stem.2023.01.006"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-09-308726"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.adg4521"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.342634.120"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.stem.2020.09.004"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-025-02692-6"},{"key":"e_1_3_2_31_2","first-page":"S157","article-title":"Genotoxic aldehyde stress prematurely ages hematopoietic stem cells in a p53-driven manner","volume":"124","author":"Wang M.","year":"2023","unstructured":"M. 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