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Esrrγa regulates nephron and ciliary development by controlling prostaglandin synthesis
Hannah M Wesselman1, Ana L Flores-Mireles2, Aidan Bauer3,4
1Department 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.
Development (Cambridge, England)
|May 26, 2023
Summary
Estrogen related receptor gamma a (ERRγa) is vital for kidney development and cilia formation in zebrafish. Its deficiency disrupts kidney patterning and cilia, but can be rescued by prostaglandin signaling, linking ERRγa to kidney health.
Area of Science:
- Developmental Biology
- Molecular Biology
- Renal Physiology
Background:
- Cilia play crucial roles in tissue development and function, particularly in the kidney.
- The transcription factor estrogen related receptor gamma a (Esrrγa) has been identified as a key regulator in various biological processes.
Purpose of the Study:
- To investigate the role of Esrrγa in renal cell fate determination and ciliogenesis in zebrafish.
- To explore the molecular mechanisms underlying Esrrγa's function in the kidney, including its interaction with prostaglandin signaling pathways.
Main Methods:
- Utilized zebrafish as a model organism to study renal development and ciliogenesis.
- Employed genetic manipulation (esrrγa deficiency) and pharmacological interventions (PGE2, Ptgs1 inhibitors) to assess phenotypic changes.
- Investigated genetic interactions between Esrrγa and Ppargc1a.
- Examined renal epithelial cell (REC)-specific ERRγ knockout mice to validate findings in a mammalian model.
Main Results:
- Esrrγa deficiency in zebrafish led to altered kidney patterning, reduced multiciliated cells, and disrupted ciliogenesis in multiple organs.
- Phenotypes were linked to prostaglandin signaling disruptions, with rescue observed upon PGE2 or Ptgs1 modulation.
- A synergistic relationship between Esrrγa and Ppargc1a in ciliogenesis was identified.
- Mice lacking REC ERRγ exhibited shorter cilia and subsequent cyst formation, indicating early ciliary dysfunction in pathogenesis.
Conclusions:
- Esrrγa is essential for renal cell fate choice and ciliogenesis in zebrafish, acting through prostaglandin signaling.
- Esrrγa cooperates with Ppargc1a in the regulation of ciliogenesis.
- Ciliary dysfunction mediated by ERRγ is an early event in kidney cystogenesis, positioning Esrrγa as a critical factor in nephrogenesis and ciliopathic disease.
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