{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T18:58:46Z","timestamp":1785437926272,"version":"3.56.0"},"reference-count":33,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Anim Models and Exp Med"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>U6 biogenesis 1 (USB1) gene mutations cause poikiloderma with neutropenia (PN), which is clinically characterized by skin hyperpigmentation, nail dysplasia, neutropenia, and an elevated risk of cancer. USB1 functions as an RNA exonuclease involved in RNA maturation and stability regulation, although its precise mechanism of action in the hematopoietic system remains unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We established a myeloid cell\u2010specific USB1 knockout mouse model (USB1fl\/fl\u2010Lyz2\u2010cre) using CRISPR\/Cas9. Using a combination of research methods, including Western blot, flow cytometry, messenger RNA (mRNA) sequencing, microRNA (miRNA) sequencing, and quantitative polymerase chain reaction (qPCR), we investigated the effects of USB1 deficiency on neutrophil development and differentiation, along with the underlying signaling molecular mechanisms.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Experimental results indicated that USB1 deficiency in mouse myeloid cells not only leads to dysregulation of miRNA expression but also interferes with the developmental, differentiation, and maturation processes of neutrophils by affecting key genes, such as IL1a, Selp, and Kilt. This impact can be traced back to the stages of myeloid progenitor cells.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>USB1 influences neutrophil maturation and myeloid progenitor cell differentiation by regulating the expression of specific miRNAs and mRNAs. This provides novel in\u00a0vivo experimental evidence for understanding the pathogenesis of PN in patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ame2.70206","type":"journal-article","created":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T12:00:06Z","timestamp":1776686406000},"page":"1353-1363","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<scp>USB1<\/scp>\n                    deficiency disrupts neutrophil maturation via\n                    <scp>RNA<\/scp>\n                    dysregulation independent of global pre\u2010\n                    <scp>mRNA<\/scp>\n                    splicing"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1815-4369","authenticated-orcid":false,"given":"Hang","family":"Li","sequence":"first","affiliation":[{"name":"State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guiying","family":"Shi","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiaming","family":"Tang","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yiying","family":"Huang","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-2188-2520","authenticated-orcid":false,"given":"Jie","family":"Wang","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuepei","family":"Lei","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0646-0812","authenticated-orcid":false,"given":"Lin","family":"Bai","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,4,20]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33455"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2009.11.014"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq371"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33600"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.abj8379"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-10-461491"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13651"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbc.2023.105139"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2012.08.031"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2015.06.046"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/wrna.1181"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.193169.112"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1080\/15476286.2015.1017240"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep15814"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri1257"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-016-2211-4"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12964-025-02353-2"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0140064"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12951-024-02902-w"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/bbb\/zbab116"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0712312105"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41556-024-01508-6"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2023.12.033"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2016.201"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-06-1734"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V78.8.1975.1975"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33807"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1128\/mbio.01599-24"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.1112596"},{"key":"e_1_2_11_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2015.183"},{"key":"e_1_2_11_32_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-01-624023"},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M114.557561"},{"key":"e_1_2_11_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2021.109139"}],"container-title":["Animal Models and Experimental Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ame2.70206","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ame2.70206","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ame2.70206","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T18:16:47Z","timestamp":1785435407000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ame2.70206"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,20]]},"references-count":33,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1002\/ame2.70206"],"URL":"https:\/\/doi.org\/10.1002\/ame2.70206","archive":["Portico"],"relation":{},"ISSN":["2576-2095","2576-2095"],"issn-type":[{"value":"2576-2095","type":"print"},{"value":"2576-2095","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,20]]},"assertion":[{"value":"2026-01-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-16","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-04-20","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}