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Norway

PMID:26469318  

RFX transcription factors are essential for hearing in mice.

Ran Elkon | Beatrice Milon | Laura Morrison | Manan Shah | Sarath Vijayakumar | Manoj Racherla | Carmen C Leitch | Lorna Silipino | Shadan Hadi | Michèle Weiss-Gayet | Emmanuèle Barras | Christoph D Schmid | Aouatef Ait-Lounis | Ashley Barnes | Yang Song | David J Eisenman | Efrat Eliyahu | Gregory I Frolenkov | Scott E Strome | Bénédicte Durand | Norann A Zaghloul | Sherri M Jones | Walter Reith | Ronna Hertzano
Nature communications | 2015

Sensorineural hearing loss is a common and currently irreversible disorder, because mammalian hair cells (HCs) do not regenerate and current stem cell and gene delivery protocols result only in immature HC-like cells. Importantly, although the transcriptional regulators of embryonic HC development have been described, little is known about the postnatal regulators of maturating HCs. Here we apply a cell type-specific functional genomic analysis to the transcriptomes of auditory and vestibular sensory epithelia from early postnatal mice. We identify RFX transcription factors as essential and evolutionarily conserved regulators of the HC-specific transcriptomes, and detect Rfx1,2,3,5 and 7 in the developing HCs. To understand the role of RFX in hearing, we generate Rfx1/3 conditional knockout mice. We show that these mice are deaf secondary to rapid loss of initially well-formed outer HCs. These data identify an essential role for RFX in hearing and survival of the terminally differentiating outer HCs.

Pubmed ID: 26469318

Research resources used in this publication

None found

Antibodies used in this publication

None found

Associated grants

  • Agency: NCRR NIH HHS, United States
    Id: S10 RR024548
  • Agency: NIDCD NIH HHS, United States
    Id: R01 DC014658
  • Agency: NIDDK NIH HHS, United States
    Id: R01DK102001
  • Agency: NIDCD NIH HHS, United States
    Id: R01 DC013817
  • Agency: NIDCD NIH HHS, United States
    Id: U01 DC013817
  • Agency: NIDCD NIH HHS, United States
    Id: R01DC03544
  • Agency: NIDDK NIH HHS, United States
    Id: P30 DK072488
  • Agency: NIDDK NIH HHS, United States
    Id: K01 DK092402
  • Agency: NIDCD NIH HHS, United States
    Id: R01DC014658
  • Agency: NIDDK NIH HHS, United States
    Id: R01 DK102001
  • Agency: NIDCD NIH HHS, United States
    Id: R01DC013817
  • Agency: NIDCD NIH HHS, United States
    Id: R01 DC003544
  • Agency: NIDDK NIH HHS, United States
    Id: K01DK092402

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