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

doi:https://doi.org/10.1038/ncomms9549

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 DurandNorann A. Zaghloul, Sherri M. Jones, Walter Reith, Ronna Hertzano

Human Molecular Genetics Biochemistry

Tel Aviv University

Research output: Contribution to journal › Article › peer-review

Abstract

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.

Original language	English
Article number	8549
Journal	Nature Communications
Volume	6
DOIs	https://doi.org/10.1038/ncomms9549
State	Published - 15 Oct 2015

All Science Journal Classification (ASJC) codes

General Chemistry
General Biochemistry,Genetics and Molecular Biology
General Physics and Astronomy

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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https://doi.org/10.1038/ncomms9549

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Elkon, R., Milon, B., Morrison, L., Shah, M., Vijayakumar, S., Racherla, M., Leitch, C. C., Silipino, L., Hadi, S., Weiss-Gayet, M., Barras, E., Schmid, C. D., Ait-Lounis, A., Barnes, A., Song, Y., Eisenman, D. J., Eliyahu, E., Frolenkov, G. I., Strome, S. E., ... Hertzano, R. (2015). RFX transcription factors are essential for hearing in mice. Nature Communications, 6, Article 8549. https://doi.org/10.1038/ncomms9549

@article{21b938d9edc54273a91697e304123c0f,

title = "RFX transcription factors are essential for hearing in mice",

abstract = "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.",

author = "Ran Elkon and Beatrice Milon and Laura Morrison and Manan Shah and Sarath Vijayakumar and Manoj Racherla and Leitch, {Carmen C.} and Lorna Silipino and Shadan Hadi and Mich{\`e}le Weiss-Gayet and Emmanu{\`e}le Barras and Schmid, {Christoph D.} and Aouatef Ait-Lounis and Ashley Barnes and Yang Song and Eisenman, {David J.} and Efrat Eliyahu and Frolenkov, {Gregory I.} and Strome, {Scott E.} and B{\'e}n{\'e}dicte Durand and Zaghloul, {Norann A.} and Jones, {Sherri M.} and Walter Reith and Ronna Hertzano",

year = "2015",

month = oct,

day = "15",

doi = "https://doi.org/10.1038/ncomms9549",

language = "الإنجليزيّة",

volume = "6",

journal = "Nature Communications",

issn = "2041-1723",

publisher = "Nature Publishing Group",

}

Elkon, R, Milon, B, Morrison, L, Shah, M, Vijayakumar, S, Racherla, M, Leitch, CC, Silipino, L, Hadi, S, Weiss-Gayet, M, Barras, E, Schmid, CD, Ait-Lounis, A, Barnes, A, Song, Y, Eisenman, DJ, Eliyahu, E, Frolenkov, GI, Strome, SE, Durand, B, Zaghloul, NA, Jones, SM, Reith, W & Hertzano, R 2015, 'RFX transcription factors are essential for hearing in mice', Nature Communications, vol. 6, 8549. https://doi.org/10.1038/ncomms9549

TY - JOUR

T1 - RFX transcription factors are essential for hearing in mice

AU - Elkon, Ran

AU - Milon, Beatrice

AU - Morrison, Laura

AU - Shah, Manan

AU - Vijayakumar, Sarath

AU - Racherla, Manoj

AU - Leitch, Carmen C.

AU - Silipino, Lorna

AU - Hadi, Shadan

AU - Weiss-Gayet, Michèle

AU - Barras, Emmanuèle

AU - Schmid, Christoph D.

AU - Ait-Lounis, Aouatef

AU - Barnes, Ashley

AU - Song, Yang

AU - Eisenman, David J.

AU - Eliyahu, Efrat

AU - Frolenkov, Gregory I.

AU - Strome, Scott E.

AU - Durand, Bénédicte

AU - Zaghloul, Norann A.

AU - Jones, Sherri M.

AU - Reith, Walter

AU - Hertzano, Ronna

PY - 2015/10/15

Y1 - 2015/10/15

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84944340041&partnerID=8YFLogxK

U2 - https://doi.org/10.1038/ncomms9549

DO - https://doi.org/10.1038/ncomms9549

M3 - مقالة

SN - 2041-1723

VL - 6

JO - Nature Communications

JF - Nature Communications

M1 - 8549

ER -

RFX transcription factors are essential for hearing in mice

Abstract

All Science Journal Classification (ASJC) codes

UN SDGs

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