Emx2 is an essential regulator of ciliated cell development across embryonic tissues

Summary: Cilia are hair-like organelles with vital physiological roles, and ciliogenesis defects underlie a range of severe congenital malformations and human diseases. Here, we report that empty spiracles homeobox 2 (emx2) is essential for cilia development across multiple embryonic tissues includi...

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Main Authors: Thanh Khoa Nguyen, John-Michael Rodriguez, Hannah M. Wesselman, Rebecca A. Wingert
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:iScience
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Online Access:http://www.sciencedirect.com/science/article/pii/S2589004224024969
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author Thanh Khoa Nguyen
John-Michael Rodriguez
Hannah M. Wesselman
Rebecca A. Wingert
author_facet Thanh Khoa Nguyen
John-Michael Rodriguez
Hannah M. Wesselman
Rebecca A. Wingert
author_sort Thanh Khoa Nguyen
collection DOAJ
description Summary: Cilia are hair-like organelles with vital physiological roles, and ciliogenesis defects underlie a range of severe congenital malformations and human diseases. Here, we report that empty spiracles homeobox 2 (emx2) is essential for cilia development across multiple embryonic tissues including the ear, neuromasts and Kupffer’s vesicle (KV), which establishes left/right axial pattern. emx2 deficient embryos manifest altered fluid homeostasis and kidney defects including decreased multiciliated cells (MCCs), determining that emx2 is essential to properly establish several renal lineages. Further, emx2 deficiency disrupted renal monociliated cells, MCCs and led to aberrant basal body positioning. We reported that emx2 regulates prostaglandin biosynthesis in ciliogenesis and renal fate changes through key factors including ppargc1a, ptgs1 and PGE2. Our findings reveal essential roles of emx2 in tissue cilia development, and identify emx2 as a critical regulator of prostaglandin biosynthesis during renal development and ciliogenesis, providing insights relevant for future treatments of ciliopathies.
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spelling doaj-art-e5afdf1c5ed446b3b5df846aa13c92452025-08-20T02:39:48ZengElsevieriScience2589-00422024-12-01271211127110.1016/j.isci.2024.111271Emx2 is an essential regulator of ciliated cell development across embryonic tissuesThanh Khoa Nguyen0John-Michael Rodriguez1Hannah M. Wesselman2Rebecca A. Wingert3Department of Biological Sciences, Center for Stem Cells and Regenerative Medicine, Center for Zebrafish Research, Boler-Parseghian Center for Rare and Neglected Diseases, Warren Center for Drug Discovery, University of Notre Dame, Notre Dame, IN 46556, USA; Corresponding authorDepartment of Biological Sciences, Center for Stem Cells and Regenerative Medicine, Center for Zebrafish Research, Boler-Parseghian Center for Rare and Neglected Diseases, Warren Center for Drug Discovery, University of Notre Dame, Notre Dame, IN 46556, USADepartment of Biological Sciences, Center for Stem Cells and Regenerative Medicine, Center for Zebrafish Research, Boler-Parseghian Center for Rare and Neglected Diseases, Warren Center for Drug Discovery, University of Notre Dame, Notre Dame, IN 46556, USADepartment of Biological Sciences, Center for Stem Cells and Regenerative Medicine, Center for Zebrafish Research, Boler-Parseghian Center for Rare and Neglected Diseases, Warren Center for Drug Discovery, University of Notre Dame, Notre Dame, IN 46556, USA; Corresponding authorSummary: Cilia are hair-like organelles with vital physiological roles, and ciliogenesis defects underlie a range of severe congenital malformations and human diseases. Here, we report that empty spiracles homeobox 2 (emx2) is essential for cilia development across multiple embryonic tissues including the ear, neuromasts and Kupffer’s vesicle (KV), which establishes left/right axial pattern. emx2 deficient embryos manifest altered fluid homeostasis and kidney defects including decreased multiciliated cells (MCCs), determining that emx2 is essential to properly establish several renal lineages. Further, emx2 deficiency disrupted renal monociliated cells, MCCs and led to aberrant basal body positioning. We reported that emx2 regulates prostaglandin biosynthesis in ciliogenesis and renal fate changes through key factors including ppargc1a, ptgs1 and PGE2. Our findings reveal essential roles of emx2 in tissue cilia development, and identify emx2 as a critical regulator of prostaglandin biosynthesis during renal development and ciliogenesis, providing insights relevant for future treatments of ciliopathies.http://www.sciencedirect.com/science/article/pii/S2589004224024969PhysiologyDevelopmental geneticsDevelopmental biology
spellingShingle Thanh Khoa Nguyen
John-Michael Rodriguez
Hannah M. Wesselman
Rebecca A. Wingert
Emx2 is an essential regulator of ciliated cell development across embryonic tissues
iScience
Physiology
Developmental genetics
Developmental biology
title Emx2 is an essential regulator of ciliated cell development across embryonic tissues
title_full Emx2 is an essential regulator of ciliated cell development across embryonic tissues
title_fullStr Emx2 is an essential regulator of ciliated cell development across embryonic tissues
title_full_unstemmed Emx2 is an essential regulator of ciliated cell development across embryonic tissues
title_short Emx2 is an essential regulator of ciliated cell development across embryonic tissues
title_sort emx2 is an essential regulator of ciliated cell development across embryonic tissues
topic Physiology
Developmental genetics
Developmental biology
url http://www.sciencedirect.com/science/article/pii/S2589004224024969
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AT johnmichaelrodriguez emx2isanessentialregulatorofciliatedcelldevelopmentacrossembryonictissues
AT hannahmwesselman emx2isanessentialregulatorofciliatedcelldevelopmentacrossembryonictissues
AT rebeccaawingert emx2isanessentialregulatorofciliatedcelldevelopmentacrossembryonictissues