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RFX transcription factors are essential for hearing in mice
Ran Elkon1, Beatrice Milon2, Laura Morrison2
1Department of Human Molecular Genetics and Biochemistry, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel.
Nature Communications
|October 16, 2015
Summary
RFX transcription factors are crucial for mature hearing. Deleting Rfx1 and Rfx3 genes in mice leads to rapid outer hair cell loss and deafness, revealing their essential role in hearing and hair cell survival.
Area of Science:
- Oto-neuroscience
- Genetics
- Developmental Biology
Background:
- Sensorineural hearing loss is irreversible due to mammalian hair cell (HC) regeneration limitations.
- Current stem cell and gene therapies yield immature HC-like cells, not functional HCs.
- Postnatal regulators of maturing HCs remain largely unknown.
Purpose of the Study:
- To identify postnatal regulators of maturing auditory and vestibular hair cells.
- To investigate the role of RFX transcription factors in HC development and hearing.
Main Methods:
- Cell type-specific functional genomic analysis of early postnatal mouse auditory and vestibular sensory epithelia transcriptomes.
- Generation and analysis of Rfx1/3 conditional knockout mice.
Main Results:
- RFX transcription factors (Rfx1, Rfx2, Rfx3, Rfx5, Rfx7) were identified as essential regulators of HC-specific transcriptomes.
- Rfx1/3 conditional knockout mice exhibited rapid outer HC loss and deafness.
- RFX factors are essential for the survival of terminally differentiating outer HCs.
Conclusions:
- RFX transcription factors play a critical role in the postnatal maturation and survival of hair cells.
- RFX factors are essential for maintaining hearing function.
- Targeting RFX pathways may offer new therapeutic strategies for hearing loss.
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