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[1]Latchman DS: Transcription factors: an overview. Int J Biochem Cell Biol 29 (12),1305-12 (1997)
[2]Bagheri-Fam S, Barrionuevo F, Dohrmann U, Günther T, Schüle R, Kemler R, et al: Long-range upstream and downstream enhancers control distinct subsets of the complex spatiotemporal Sox9 expression pattern. Dev Biol 291 (2),382-97 (2006)
[3]Sajan SA, Rubenstein JL, Warchol ME, Lovett M: Identification of direct downstream targets of Dlx5 during early inner ear development. Hum Mol Genet 20 (7),1262-73 (2011)
[4]Trowe MO, Maier H, Schweizer M, Kispert A: Deafness in mice lacking the T-box transcription factor Tbx18 in otic fibrocytes. Development 135 (9),1725-34 (2008)
[5]Monks DC, Morrow BE: Identification of putative retinoic acid target genes downstream of mesenchymal Tbx1 during inner ear development. Dev Dyn 241 (3),563-73 (2012)
[6]Engelen E, Akinci U, Bryne JC, Hou J, Gontan C, Moen M, et al: Sox2 cooperates with Chd7 to regulate genes that are mutated in human syndromes. Nat Genet 43 (6),607-11 (2011)
[7]Pilipenko VV, Reece A, Choo DI, Greinwald JH Jr: Genomic organization and expression analysis of the murine Fam3c gene. Gene 335,159-68 (2004)
[8]Hudspeth AJ, Choe Y, Mehta AD, Martin P: Putting ion channels to work: mechanoelectrical transduction, adaptation, and amplification by hair cells. Proc Natl Acad Sci U S A 97 (22),11765-72 (2000)
[9]Hackney CM, Furness DN: The composition and role of cross links in mechanoelectrical transduction in vertebrate sensory hair cells. J Cell Sci 126 (Pt 8),1721-31 (2013)
[10]Rubel EW, Furrer SA, Stone JS: A brief history of hair cell regeneration research and speculations on the future. Hear Res 297,42-51 (2013)
[11]Barald KF, Kelley MW: From placode to polarization: new tunes in inner ear development. Development 131 (17),4119-30 (2004)
[12]Groves AK, Fekete DM: Shaping sound in space: the regulation of inner ear patterning. Development 139 (2),245-57 (2012)
[13]Liu D, Chu H, Maves L, Yan YL, Morcos PA, Postlethwait JH, Westerfield M: Fgf3 and Fgf8 dependent and independent transcription factors are required for otic placode specification. Development 130 (10),2213-24 (2003)
[14]Freter S, Muta Y, O’Neill P, Vassilev VS, Kuraku S, Ladher RK: Pax2 modulates proliferation during specification of the otic and epibranchial placodes. Dev Dyn 241 (11),1716-28 (2012)
[15]Jayasena CS, Ohyama T, Segil N, Groves AK: Notch signaling augments the canonical Wnt pathway to specify the size of the otic placode. Development 135 (13),2251-61 (2008)
[16]Barrionuevo F, Naumann A, Bagheri-Fam S, Speth V, Taketo MM, Scherer G, Neubüser A: Sox9 is required for invagination of the otic placode in mice. Dev Biol 317 (1),213-24 (2008)
[17]Park BY, Saint-Jeannet JP: Long-term consequences of Sox9 depletion on inner ear development. Dev Dyn 239 (4),1102-12 (2010)
[18]Ponnio T, Burton Q, Pereira FA, Wu DK, Conneely OM: The nuclear receptor Nor-1 is essential for proliferation of the semicircular canals of the mouse inner ear. Mol Cell Biol 22 (3),935-45 (2002)
[19]Lin Z, Cantos R, Patente M, Wu DK: Gbx2 is required for the morphogenesis of the mouse inner ear: a downstream candidate of hindbrain signaling. Development 132 (10),2309-18 (2005)
[20]Liu M, Pereira FA, Price SD, Chu MJ, Shope C, Himes D, et al. Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems. Genes Dev 14 (22),2839-54 (2000)
[21]Hulander M, Wurst W, Carlsson P, Enerbäck S: The winged helix transcription factor Fkh10 is required for normal development of the inner ear. Nat Genet 20 (4),374-6 (1998)
[22]Warchol ME, Richardson GP: Expression of the Pax2 transcription factor is associated with vestibular phenotype in the avian inner ear. Dev Neurobiol 69 (2-3),191-202 (2009)
[23]Hutson MR, Lewis JE, Nguyen-Luu D, Lindberg KH, Barald KF: Expression of Pax2 and patterning of the chick inner ear. J Neurocytol 28 (10-11),795-807 (1999)
[24]Burton Q, Cole LK, Mulheisen M, Chang W, Wu DK: The role of Pax2 in mouse inner ear development. Dev Biol 272 (1),161-75 (2004)
[25]Braunstein EM, Crenshaw EB 3rd, Morrow BE, Adams JC: Cooperative function of Tbx1 and Brn4 in the periotic mesenchyme is necessary for cochlea formation. J Assoc Res Otolaryngol 9 (1),33-43 (2008)
[26]Sanchez-Calderon H, Rodriguez-de la Rosa L, Milo M, Pichel JG, Holley M, Varela-Nieto I: RNA microarray analysis in prenatal mouse cochlea reveals novel IGF-I target genes: implication of MEF2 and FOXM1 transcription factors. PLoS One 5 (1),e8699 (2010)
[27]Aburto MR, Magariños M, Leon Y, Varela-Nieto I, Sanchez-Calderon H: AKT signaling mediates IGF-I survival actions on otic neural progenitors. PLoS One 7 (1),e30790 (2012)
[28]Yamamoto N, Chang W, Kelley MW: Rbpj regulates development of prosensory cells in the mammalian inner ear. Dev Biol 353(2),367-79 (2011)
[29]Driver EC, Pryor SP, Hill P, Turner J, Rüther U, Biesecker LG, et al: Hedgehog signaling regulates sensory cell formation and auditory function in mice and humans. J Neurosci 28 (29),7350-8 (2008)
[30]Khatri SB, Groves AK: Expression of the Foxi2 and Foxi3 transcription factors during development of chicken sensory placodes and pharyngeal arches. Gene Expr Patterns 13 (1-2),38-42 (2013)
[31]Nie X, Zhang K, Wang L, Ou G, Zhu H, Gao WQ: Transcription factor STOX1 regulates proliferation of inner ear epithelial cells via the AKT pathway. Cell Prolif 48 (2),209-20 (2015)
[32]Kantarci H, Edlund RK, Groves AK, Riley BB: Tfap2a promotes specification and maturation of neurons in the inner ear through modulation of Bmp, Fgf and notch signaling. PLoS Genet 11 (3),e1005037 (2015)
[33]Xiang M, Gan L, Li D, Chen ZY, Zhou L, O’Malley BW Jr, et al: Essential role of POU-domain factor Brn-3c in auditory and vestibular hair cell development. Proc Natl Acad Sci U S A 94 (17),9445-50 (1997)
[34]Kelley MW, Driver EC, Puligilla C: Regulation of cell fate and patterning in the developing mammalian cochlea. Curr Opin Otolaryngol Head Neck Surg 17 (5),381-7 (2009)
[35]Roberson DW, Alosi JA, Mercola M, Cotanche DA: REST mRNA expression in normal and regenerating avian auditory epithelium. Hear Res 172 (1-2),62-72 (2002)
[36]Bricaud O, Collazo A: The transcription factor six1 inhibits neuronal and promotes hair cell fate in the developing zebrafish (Danio rerio) inner ear. J Neurosci 26 (41),10438-51 (2006)
[37]Pechriggl EJ, Bitsche M, Glueckert R, Rask-Andersen H, Blumer MJ, Schrott-Fischer A, Fritsch H: Development of the innervation of the human inner ear. Dev Neurobiol 75 (7),683-702 (2015)
[38]Smeti I, Watabe I, Savary E, Fontbonne A, Zine A: HMGA2, the architectural transcription factor high mobility group, is expressed in the developing and mature mouse cochlea. PLoS One 9 (2),e88757 (2014)
[39]Atar O, Avraham KB: Anti-apoptotic factor z-Val-Ala-Asp-fluoromethylketone promotes the survival of cochlear hair cells in a mouse model for human deafness. Neuroscience 168 (3),851-7 (2010)
[40]Zhuang S, Zhang Q, Zhuang T, Evans SM, Liang X, Sun Y: Expression of Isl1 during mouse development. Gene Expr Patterns 13 (8),407-12 (2013)
[41]Huang M, Kantardzhieva A, Scheffer D, Liberman MC, Chen ZY: Hair cell overexpression of Islet1 reduces age-related and noise-induced hearing loss. J Neurosci 33 (38),15086-94 (2013)
[42]Wallis D, Hamblen M, Zhou Y, Venken KJ, Schumacher A, Grimes HL, et al: The zinc finger transcription factor Gfi1, implicated in lymphomagenesis, is required for inner ear hair cell differentiation and survival. Development 130 (1),221-32 (2003)
[43]Hertzano R, Montcouquiol M, Rashi-Elkeles S, Elkon R, Yücel R, Frankel WN, et al: Transcription profiling of inner ears from Pou4f3 (ddl/ddl) identifies Gfi1 as a target of the Pou4f3 deafness gene. Hum Mol Genet 13 (18),2143-53 (2004)
[44]Tornari C, Towers ER, Gale JE, Dawson SJ: Regulation of the orphan nuclear receptor Nr2f2 by the DFNA15 deafness gene Pou4f3. PLoS One 9(11),e112247 (2014)
[45]Bermingham NA, Hassan BA, Price SD, Vollrath MA, Ben-Arie N, Eatock RA, et al: Math1: an essential gene for the generation of inner ear hair cells. Science 284 (5421),1837-41 (1999)
[46]Bermingham NA, Hassan BA, Wang VY, Fernandez M, Banfi S, Bellen HJ, et al: Proprioceptor pathway development is dependent on Math1. Neuron 30 (2), 411-22 (2001)
[47]Woods C, Montcouquiol M, Kelley MW: Math1 regulates development of the sensory epithelium in the mammalian cochlea. Nat Neurosci 7(12),1310-8 (2004)
[48]Ouji Y, Ishizaka S, Nakamura-Uchiyama F, Wanaka A, Yoshikawa M: Induction of inner ear hair cell-like cells from Math1-transfected mouse ES cells. Cell Death Dis 4,e700 (2013)
[49]Yang XY, Jin K, Ma R, Yang JM, Luo WW, Han Z, et al: Role of the planar cell polarity pathway in regulating ectopic hair cell-like cells induced by Math1 and testosterone treatment. Brain Res 1615,22-30 (2015)
[50]Kiernan AE, Pelling AL, Leung KK, Tang AS, Bell DM, Tease C, et al: Sox2 is required for sensory organ development in the mammalian inner ear. Nature 434 (7036),1031-5 (2005)
[51]Neves J, Kamaid A, Alsina B, Giraldez F: Differential expression of Sox2 and Sox3 in neuronal and sensory progenitors of the developing inner ear of the chick. J Comp Neurol 503 (4),487-500 (2007)
[52]Pan W, Jin Y, Chen J, Rottier RJ, Steel KP, Kiernan AE: Ectopic expression of activated notch or SOX2 reveals similar and unique roles in the development of the sensory cell progenitors in the mammalian inner ear. J Neurosci 33 (41),16146-57 (2013)
[53]Dabdoub A, Puligilla C, Jones JM, Fritzsch B, Cheah KS, Pevny LH, et al: Sox2 signaling in prosensory domain specification and subsequent hair cell differentiation in the developing cochlea. Proc Natl Acad Sci U S A 105 (47),18396-401 (2008)
[54]Neves J, Uchikawa M, Bigas A, Giraldez F: The prosensory function of Sox2 in the chicken inner ear relies on the direct regulation of Atoh1. PLoS One 7 (1),e30871 (2012)
[55]Yang H, Xie X, Deng M, Chen X, Gan L: Generation and characterization of Atoh1-Cre knock-in mouse line. Genesis 48 (6),407-13 (2010)
[56]Pan N, Jahan I, Kersigo J, Kopecky B, Santi P, Johnson S, et al: Conditional deletion of Atoh1 using Pax2-Cre results in viable mice without differentiated cochlear hair cells that have lost most of the organ of Corti. Hear Res 275(1-2),66-80 (2011)
[57]Sheykholeslami K, Thimmappa V, Nava C, Bai X, Yu H, Zheng T, et al: Anew mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. PLoS One 8 (11),e79791 (2013)
[58]Pan N, Jahan I, Kersigo J, Duncan JS, Kopecky B, Fritzsch B: Anovel Atoh1 “self-terminating” mouse model reveals the necessity of proper Atoh1 level and duration for hair cell differentiation and viability. PLoS One 7 (1),e30358 (2012)
[59]Lin Z, Perez P, Sun Z, Liu JJ, Shin JH, Hyrc KL, et al: Reprogramming of single-cell-derived mesenchymal stem cells into hair cell-like cells. Otol Neurotol 33 (9),1648-55 (2012)
[60]Kouros-Mehr H, Slorach EM, Sternlicht MD, Werb Z: GATA-3 maintains the differentiation of the luminal cell fate in the mammary gland. Cell 127 (5),1041-55 (2006)
[61]Yagi R, Zhu J, Paul WE: An updated view on transcription factor GATA3-mediated regulation of Th1 and Th2 cell differentiation. Int Immunol 23 (7),415-20 (2011)
[62]van der Wees J, van Looij MA, de Ruiter MM, Elias H, van der Burg H, et al: Hearing loss following Gata3 haploinsufficiency is caused by cochlear disorder. Neurobiol Dis 16 (1),169-78 (2004)
[63]Milo M, Cacciabue-Rivolta D, Kneebone A, Van Doorninck H, Johnson C, Lawoko-KeraliG, et al: Genomic analysis of the function of the transcription factor gata3 during development of the mammalian inner ear. PLoS One 4 (9):e7144 (2009)
[64]Duncan JS, Fritzsch B: Continued expression of GATA3 is necessary for cochlear neurosensory development. PLoS One 8(4):e62046 (2013)
[65]Rivolta MN, Holley MC: GATA3 is downregulated during hair cell differentiation in the mouse cochlea. J Neurocytol 27(9):637-47 (1998)
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Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.
Transcription factors in the development of inner ear hair cells
1 Department of Otolaryngology and Head-Neck Surgery, Zhenjiang First People’s Hospital, The Affiliated People’s Hospital of Jiangsu University, Zhenjiang, Jiangsu, China
Abstract
Inner ear hair cells are the sensory receptors that detect and convert sound vibrations and head movements into neural signals. However, in humans, these cells are unable to regenerate if they are damaged or lost. Over thepast decade,there has been an exponential increase in interest and progress in understanding of the development of the inner ear and of hair cells, aiming to gain insights into hair cell repair or even regeneration. In hair cell development, various transcription factors have been found to be involved in the processes of hair cell proliferation, differentiation and survival. Among these transcription factors, Math1, Gata3, Sox2 and Atoh1 have been highlighted for their crucial role in the fate of hair cells. In this article, we will summarize the current understanding of the role of transcription factors in hair cell development, focusing on the role and possible mechanisms of Math1, Gata3, Sox2 and Atoh1.
Keywords
- Inner Ear
- Hair Cell
- Transcription Factor
- Review
References
- [1] Latchman DS: Transcription factors: an overview. Int J Biochem Cell Biol 29 (12),1305-12 (1997)
- [2] Bagheri-Fam S, Barrionuevo F, Dohrmann U, Günther T, Schüle R, Kemler R, et al: Long-range upstream and downstream enhancers control distinct subsets of the complex spatiotemporal Sox9 expression pattern. Dev Biol 291 (2),382-97 (2006)
- [3] Sajan SA, Rubenstein JL, Warchol ME, Lovett M: Identification of direct downstream targets of Dlx5 during early inner ear development. Hum Mol Genet 20 (7),1262-73 (2011)
- [4] Trowe MO, Maier H, Schweizer M, Kispert A: Deafness in mice lacking the T-box transcription factor Tbx18 in otic fibrocytes. Development 135 (9),1725-34 (2008)
- [5] Monks DC, Morrow BE: Identification of putative retinoic acid target genes downstream of mesenchymal Tbx1 during inner ear development. Dev Dyn 241 (3),563-73 (2012)
- [6] Engelen E, Akinci U, Bryne JC, Hou J, Gontan C, Moen M, et al: Sox2 cooperates with Chd7 to regulate genes that are mutated in human syndromes. Nat Genet 43 (6),607-11 (2011)
- [7] Pilipenko VV, Reece A, Choo DI, Greinwald JH Jr: Genomic organization and expression analysis of the murine Fam3c gene. Gene 335,159-68 (2004)
- [8] Hudspeth AJ, Choe Y, Mehta AD, Martin P: Putting ion channels to work: mechanoelectrical transduction, adaptation, and amplification by hair cells. Proc Natl Acad Sci U S A 97 (22),11765-72 (2000)
- [9] Hackney CM, Furness DN: The composition and role of cross links in mechanoelectrical transduction in vertebrate sensory hair cells. J Cell Sci 126 (Pt 8),1721-31 (2013)
- [10] Rubel EW, Furrer SA, Stone JS: A brief history of hair cell regeneration research and speculations on the future. Hear Res 297,42-51 (2013)
- [11] Barald KF, Kelley MW: From placode to polarization: new tunes in inner ear development. Development 131 (17),4119-30 (2004)
- [12] Groves AK, Fekete DM: Shaping sound in space: the regulation of inner ear patterning. Development 139 (2),245-57 (2012)
- [13] Liu D, Chu H, Maves L, Yan YL, Morcos PA, Postlethwait JH, Westerfield M: Fgf3 and Fgf8 dependent and independent transcription factors are required for otic placode specification. Development 130 (10),2213-24 (2003)
- [14] Freter S, Muta Y, O’Neill P, Vassilev VS, Kuraku S, Ladher RK: Pax2 modulates proliferation during specification of the otic and epibranchial placodes. Dev Dyn 241 (11),1716-28 (2012)
- [15] Jayasena CS, Ohyama T, Segil N, Groves AK: Notch signaling augments the canonical Wnt pathway to specify the size of the otic placode. Development 135 (13),2251-61 (2008)
- [16] Barrionuevo F, Naumann A, Bagheri-Fam S, Speth V, Taketo MM, Scherer G, Neubüser A: Sox9 is required for invagination of the otic placode in mice. Dev Biol 317 (1),213-24 (2008)
- [17] Park BY, Saint-Jeannet JP: Long-term consequences of Sox9 depletion on inner ear development. Dev Dyn 239 (4),1102-12 (2010)
- [18] Ponnio T, Burton Q, Pereira FA, Wu DK, Conneely OM: The nuclear receptor Nor-1 is essential for proliferation of the semicircular canals of the mouse inner ear. Mol Cell Biol 22 (3),935-45 (2002)
- [19] Lin Z, Cantos R, Patente M, Wu DK: Gbx2 is required for the morphogenesis of the mouse inner ear: a downstream candidate of hindbrain signaling. Development 132 (10),2309-18 (2005)
- [20] Liu M, Pereira FA, Price SD, Chu MJ, Shope C, Himes D, et al. Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems. Genes Dev 14 (22),2839-54 (2000)
- [21] Hulander M, Wurst W, Carlsson P, Enerbäck S: The winged helix transcription factor Fkh10 is required for normal development of the inner ear. Nat Genet 20 (4),374-6 (1998)
- [22] Warchol ME, Richardson GP: Expression of the Pax2 transcription factor is associated with vestibular phenotype in the avian inner ear. Dev Neurobiol 69 (2-3),191-202 (2009)
- [23] Hutson MR, Lewis JE, Nguyen-Luu D, Lindberg KH, Barald KF: Expression of Pax2 and patterning of the chick inner ear. J Neurocytol 28 (10-11),795-807 (1999)
- [24] Burton Q, Cole LK, Mulheisen M, Chang W, Wu DK: The role of Pax2 in mouse inner ear development. Dev Biol 272 (1),161-75 (2004)
- [25] Braunstein EM, Crenshaw EB 3rd, Morrow BE, Adams JC: Cooperative function of Tbx1 and Brn4 in the periotic mesenchyme is necessary for cochlea formation. J Assoc Res Otolaryngol 9 (1),33-43 (2008)
- [26] Sanchez-Calderon H, Rodriguez-de la Rosa L, Milo M, Pichel JG, Holley M, Varela-Nieto I: RNA microarray analysis in prenatal mouse cochlea reveals novel IGF-I target genes: implication of MEF2 and FOXM1 transcription factors. PLoS One 5 (1),e8699 (2010)
- [27] Aburto MR, Magariños M, Leon Y, Varela-Nieto I, Sanchez-Calderon H: AKT signaling mediates IGF-I survival actions on otic neural progenitors. PLoS One 7 (1),e30790 (2012)
- [28] Yamamoto N, Chang W, Kelley MW: Rbpj regulates development of prosensory cells in the mammalian inner ear. Dev Biol 353(2),367-79 (2011)
- [29] Driver EC, Pryor SP, Hill P, Turner J, Rüther U, Biesecker LG, et al: Hedgehog signaling regulates sensory cell formation and auditory function in mice and humans. J Neurosci 28 (29),7350-8 (2008)
- [30] Khatri SB, Groves AK: Expression of the Foxi2 and Foxi3 transcription factors during development of chicken sensory placodes and pharyngeal arches. Gene Expr Patterns 13 (1-2),38-42 (2013)
- [31] Nie X, Zhang K, Wang L, Ou G, Zhu H, Gao WQ: Transcription factor STOX1 regulates proliferation of inner ear epithelial cells via the AKT pathway. Cell Prolif 48 (2),209-20 (2015)
- [32] Kantarci H, Edlund RK, Groves AK, Riley BB: Tfap2a promotes specification and maturation of neurons in the inner ear through modulation of Bmp, Fgf and notch signaling. PLoS Genet 11 (3),e1005037 (2015)
- [33] Xiang M, Gan L, Li D, Chen ZY, Zhou L, O’Malley BW Jr, et al: Essential role of POU-domain factor Brn-3c in auditory and vestibular hair cell development. Proc Natl Acad Sci U S A 94 (17),9445-50 (1997)
- [34] Kelley MW, Driver EC, Puligilla C: Regulation of cell fate and patterning in the developing mammalian cochlea. Curr Opin Otolaryngol Head Neck Surg 17 (5),381-7 (2009)
- [35] Roberson DW, Alosi JA, Mercola M, Cotanche DA: REST mRNA expression in normal and regenerating avian auditory epithelium. Hear Res 172 (1-2),62-72 (2002)
- [36] Bricaud O, Collazo A: The transcription factor six1 inhibits neuronal and promotes hair cell fate in the developing zebrafish (Danio rerio) inner ear. J Neurosci 26 (41),10438-51 (2006)
- [37] Pechriggl EJ, Bitsche M, Glueckert R, Rask-Andersen H, Blumer MJ, Schrott-Fischer A, Fritsch H: Development of the innervation of the human inner ear. Dev Neurobiol 75 (7),683-702 (2015)
- [38] Smeti I, Watabe I, Savary E, Fontbonne A, Zine A: HMGA2, the architectural transcription factor high mobility group, is expressed in the developing and mature mouse cochlea. PLoS One 9 (2),e88757 (2014)
- [39] Atar O, Avraham KB: Anti-apoptotic factor z-Val-Ala-Asp-fluoromethylketone promotes the survival of cochlear hair cells in a mouse model for human deafness. Neuroscience 168 (3),851-7 (2010)
- [40] Zhuang S, Zhang Q, Zhuang T, Evans SM, Liang X, Sun Y: Expression of Isl1 during mouse development. Gene Expr Patterns 13 (8),407-12 (2013)
- [41] Huang M, Kantardzhieva A, Scheffer D, Liberman MC, Chen ZY: Hair cell overexpression of Islet1 reduces age-related and noise-induced hearing loss. J Neurosci 33 (38),15086-94 (2013)
- [42] Wallis D, Hamblen M, Zhou Y, Venken KJ, Schumacher A, Grimes HL, et al: The zinc finger transcription factor Gfi1, implicated in lymphomagenesis, is required for inner ear hair cell differentiation and survival. Development 130 (1),221-32 (2003)
- [43] Hertzano R, Montcouquiol M, Rashi-Elkeles S, Elkon R, Yücel R, Frankel WN, et al: Transcription profiling of inner ears from Pou4f3 (ddl/ddl) identifies Gfi1 as a target of the Pou4f3 deafness gene. Hum Mol Genet 13 (18),2143-53 (2004)
- [44] Tornari C, Towers ER, Gale JE, Dawson SJ: Regulation of the orphan nuclear receptor Nr2f2 by the DFNA15 deafness gene Pou4f3. PLoS One 9(11),e112247 (2014)
- [45] Bermingham NA, Hassan BA, Price SD, Vollrath MA, Ben-Arie N, Eatock RA, et al: Math1: an essential gene for the generation of inner ear hair cells. Science 284 (5421),1837-41 (1999)
- [46] Bermingham NA, Hassan BA, Wang VY, Fernandez M, Banfi S, Bellen HJ, et al: Proprioceptor pathway development is dependent on Math1. Neuron 30 (2), 411-22 (2001)
- [47] Woods C, Montcouquiol M, Kelley MW: Math1 regulates development of the sensory epithelium in the mammalian cochlea. Nat Neurosci 7(12),1310-8 (2004)
- [48] Ouji Y, Ishizaka S, Nakamura-Uchiyama F, Wanaka A, Yoshikawa M: Induction of inner ear hair cell-like cells from Math1-transfected mouse ES cells. Cell Death Dis 4,e700 (2013)
- [49] Yang XY, Jin K, Ma R, Yang JM, Luo WW, Han Z, et al: Role of the planar cell polarity pathway in regulating ectopic hair cell-like cells induced by Math1 and testosterone treatment. Brain Res 1615,22-30 (2015)
- [50] Kiernan AE, Pelling AL, Leung KK, Tang AS, Bell DM, Tease C, et al: Sox2 is required for sensory organ development in the mammalian inner ear. Nature 434 (7036),1031-5 (2005)
- [51] Neves J, Kamaid A, Alsina B, Giraldez F: Differential expression of Sox2 and Sox3 in neuronal and sensory progenitors of the developing inner ear of the chick. J Comp Neurol 503 (4),487-500 (2007)
- [52] Pan W, Jin Y, Chen J, Rottier RJ, Steel KP, Kiernan AE: Ectopic expression of activated notch or SOX2 reveals similar and unique roles in the development of the sensory cell progenitors in the mammalian inner ear. J Neurosci 33 (41),16146-57 (2013)
- [53] Dabdoub A, Puligilla C, Jones JM, Fritzsch B, Cheah KS, Pevny LH, et al: Sox2 signaling in prosensory domain specification and subsequent hair cell differentiation in the developing cochlea. Proc Natl Acad Sci U S A 105 (47),18396-401 (2008)
- [54] Neves J, Uchikawa M, Bigas A, Giraldez F: The prosensory function of Sox2 in the chicken inner ear relies on the direct regulation of Atoh1. PLoS One 7 (1),e30871 (2012)
- [55] Yang H, Xie X, Deng M, Chen X, Gan L: Generation and characterization of Atoh1-Cre knock-in mouse line. Genesis 48 (6),407-13 (2010)
- [56] Pan N, Jahan I, Kersigo J, Kopecky B, Santi P, Johnson S, et al: Conditional deletion of Atoh1 using Pax2-Cre results in viable mice without differentiated cochlear hair cells that have lost most of the organ of Corti. Hear Res 275(1-2),66-80 (2011)
- [57] Sheykholeslami K, Thimmappa V, Nava C, Bai X, Yu H, Zheng T, et al: Anew mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. PLoS One 8 (11),e79791 (2013)
- [58] Pan N, Jahan I, Kersigo J, Duncan JS, Kopecky B, Fritzsch B: Anovel Atoh1 “self-terminating” mouse model reveals the necessity of proper Atoh1 level and duration for hair cell differentiation and viability. PLoS One 7 (1),e30358 (2012)
- [59] Lin Z, Perez P, Sun Z, Liu JJ, Shin JH, Hyrc KL, et al: Reprogramming of single-cell-derived mesenchymal stem cells into hair cell-like cells. Otol Neurotol 33 (9),1648-55 (2012)
- [60] Kouros-Mehr H, Slorach EM, Sternlicht MD, Werb Z: GATA-3 maintains the differentiation of the luminal cell fate in the mammary gland. Cell 127 (5),1041-55 (2006)
- [61] Yagi R, Zhu J, Paul WE: An updated view on transcription factor GATA3-mediated regulation of Th1 and Th2 cell differentiation. Int Immunol 23 (7),415-20 (2011)
- [62] van der Wees J, van Looij MA, de Ruiter MM, Elias H, van der Burg H, et al: Hearing loss following Gata3 haploinsufficiency is caused by cochlear disorder. Neurobiol Dis 16 (1),169-78 (2004)
- [63] Milo M, Cacciabue-Rivolta D, Kneebone A, Van Doorninck H, Johnson C, Lawoko-KeraliG, et al: Genomic analysis of the function of the transcription factor gata3 during development of the mammalian inner ear. PLoS One 4 (9):e7144 (2009)
- [64] Duncan JS, Fritzsch B: Continued expression of GATA3 is necessary for cochlear neurosensory development. PLoS One 8(4):e62046 (2013)
- [65] Rivolta MN, Holley MC: GATA3 is downregulated during hair cell differentiation in the mouse cochlea. J Neurocytol 27(9):637-47 (1998)
