eprintid: 10069932 rev_number: 24 eprint_status: archive userid: 608 dir: disk0/10/06/99/32 datestamp: 2019-03-12 09:22:33 lastmod: 2021-12-13 01:40:38 status_changed: 2019-03-12 09:22:33 type: article metadata_visibility: show creators_name: Dupays, L creators_name: Towers, N creators_name: Wood, S creators_name: David, A creators_name: Stuckey, DJ creators_name: Mohun, T title: Furin, a transcriptional target of NKX2-5, has an essential role in heart development and function ispublished: pub divisions: UCL divisions: B02 divisions: C10 divisions: D17 divisions: FI6 divisions: D11 divisions: G12 note: © 2019 Dupays et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. http://creativecommons.org/licenses/by/4.0/ abstract: The homeodomain transcription factor NKX2-5 is known to be essential for both normal heart development and for heart function. But little is yet known about the identities of its downstream effectors or their function during differentiation of cardiac progenitor cells (CPCs). We have used transgenic analysis and CRISPR-mediated ablation to identify a cardiac enhancer of the Furin gene. The Furin gene, encoding a proprotein convertase, is directly repressed by NKX2-5. Deletion of Furin in CPCs is embryonic lethal, with mutant hearts showing a range of abnormalities in the outflow tract. Those defects are associated with a reduction in proliferation and premature differentiation of the CPCs. Deletion of Furin in differentiated cardiomyocytes results in viable adult mutant mice showing an elongation of the PR interval, a phenotype that is consistent with the phenotype of mice and human mutant for Nkx2-5. Our results show that Furin mediate some aspects of Nkx2-5 function in the heart. date: 2019 date_type: published official_url: https://doi.org/10.1371/journal.pone.0212992 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green article_type_text: Journal Article verified: verified_manual elements_id: 1637933 doi: 10.1371/journal.pone.0212992 pii: PONE-D-18-26720 lyricists_name: David, Anna lyricists_name: Stuckey, Daniel lyricists_id: ADAVI52 lyricists_id: DSTUC39 actors_name: Joyce, Sophia actors_id: SJOYC12 actors_role: owner full_text_status: public publication: PLoS One volume: 14 number: 3 article_number: e0212992 event_location: United States issn: 1932-6203 citation: Dupays, L; Towers, N; Wood, S; David, A; Stuckey, DJ; Mohun, T; (2019) Furin, a transcriptional target of NKX2-5, has an essential role in heart development and function. PLoS One , 14 (3) , Article e0212992. 10.1371/journal.pone.0212992 <https://doi.org/10.1371/journal.pone.0212992>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10069932/1/Furin%2C%20a%20transcriptional%20target%20of%20NKX2-5%2C%20has%20an%20essential%20role%20in%20heart%20development%20and%20function.pdf