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While current treatments alleviate symptoms, they fail to address the underlying cause of the disease\u2014the pathogenic PNH clones. In this study, we found that the expression of carbamoyl phosphate synthetase 1 (CPS1) was downregulated in PNH clones, and the level of CPS1 was negatively correlated with the proportion of PNH clones. Using <jats:italic>PIG\u2010A<\/jats:italic> knockout K562 (K562 KO) cells, we demonstrated that CPS1 knockdown increased cell proliferation and altered cell metabolism, suggesting that CPS1 participates in PNH clonal proliferation through metabolic reprogramming. Furthermore, we observed an increase in the expression levels of the histone demethylase JMJD1C in PNH clones, and JMJD1C expression was negatively correlated with CPS1 expression. Knocking down JMJD1C in K562 KO cells upregulated CPS1 and H3K36me3 expression, decreased cell proliferation and increased cell apoptosis. Chromatin immunoprecipitation analysis further demonstrated that H3K36me3 regulated CPS1 expression. Finally, we demonstrated that histone demethylase inhibitor JIB\u201004 can suppressed K562 KO cell proliferation and reduced the proportion of PNH clones in PNH mice. In conclusion, aberrant regulation of the JMJD1C\u2010H3K36me3\u2010CPS1 axis contributes to PNH clonal proliferation. Targeting JMJD1C with a specific inhibitor unveils a potential strategy for treating PNH patients.<\/jats:p>","DOI":"10.1111\/bjh.19477","type":"journal-article","created":{"date-parts":[[2024,4,23]],"date-time":"2024-04-23T02:00:09Z","timestamp":1713837609000},"page":"2468-2479","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["The histone demethylase <scp>JMJD1C<\/scp> regulates <scp>CPS1<\/scp> expression and promotes the proliferation of paroxysmal nocturnal haemoglobinuria clones through cell metabolic reprogramming"],"prefix":"10.1111","volume":"204","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7575-0867","authenticated-orcid":false,"given":"Yingying","family":"Chen","sequence":"first","affiliation":[{"name":"Department of Hematology Tianjin Medical University General Hospital  Tianjin China"},{"name":"Tianjin Key Laboratory of Bone Marrow Failure and Malignant Hemopoietic Clone Control  Tianjin 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