Differentiation of the Lateral Compartment of the Cochlea Requires a Temporally Restricted FGF20 Signal

Huh, Sung Ho; Jones, Jennifer M.; Warchol, Mark E.; Ornitz, David M.

doi:10.1371/journal.pbio.1001231

Cited by 100 publications

(194 citation statements)

References 41 publications

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“…In addition, we tested Fgfr3 P244R/+ mice in which one copy of Fgf20 was removed. Fgf20 encodes another potential FGFR3c ligand (Zhang et al 2006) that is expressed in the developing prosensory domain and is required for outer hair cell differentiation (Hayashi et al 2008;Huh et al 2012). Even in a mixed genetic background, Fgfr3 P244R/+ ;Fgf8 +/+ ;Fgf9 +/+ mice had hearing loss that was 100% penetrant, mild-moderate in degree (30-40 dB), and more severe (40 dB) at low frequencies than at high (30 dB) (Fig.…”

Section: Fgf Signaling Expands Laterally In Muenke Syndrome Model Cocmentioning

confidence: 99%

“…Fgfr3 À/+ mice (Fgfr3 tm1Dor ; MGI: 1931521) were described by Colvin et al (1996). Fgf20 À/+ mice (Fgf20 tm1.1Dor ; MGI: 5425761) were described by Huh et al (2012). The Fgf3 À/+ (Fgf3 tm1.1Sms ; MGI: 3767558) and Fgf10 À/+ (Fgf10 tm1.1Sms ; MGI: 3526181) strains were generated and described previously (Urness et al 2010).…”

Section: Mutant Mice and Genotypingmentioning

confidence: 99%

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Genetic rescue of Muenke syndrome model hearing loss reveals prolonged FGF-dependent plasticity in cochlear supporting cell fates

Mansour

Urness

2013

Genes Dev.

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The stereotyped arrangement of cochlear sensory and supporting cells is critical for auditory function. Our previous studies showed that Muenke syndrome model mice (Fgfr3 P244R/+ ) have hearing loss associated with a supporting cell fate transformation of two Deiters' cells to two pillar cells. We investigated the developmental origins of this transformation and found that two prospective Deiters' cells switch to an outer pillar cell-like fate sequentially between embryonic day 17.5 (E17.5) and postnatal day 3 (P3). Unexpectedly, the Fgfr3 P244R/+ hearing loss and supporting cell fate transformation are not rescued by genetically reducing fibroblast growth factor 8 (FGF8), the FGF receptor 3c (FGFR3c) ligand required for pillar cell differentiation. Rather, reducing FGF10, which normally activates FGFR2b or FGFR1b, is sufficient for rescue of cochlear form and function. Accordingly, we found that the P244R mutation changes the specificity of FGFR3b and FGFR3c such that both acquire responsiveness to FGF10. Moreover, Fgf10 heterozygosity does not block the Fgfr3 P244R/+ supporting cell fate transformation but instead allows a gradual reversion of fate-switched cells toward the normal phenotype between P5 and at least P14. This study indicates that Deiters' and pillar cells can reversibly switch fates in an FGFdependent manner over a prolonged period of time. This property might be exploited for the regulation of sensory cell regeneration from support cells.

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Section: Fgf Signaling Expands Laterally In Muenke Syndrome Model Cocmentioning

confidence: 99%

Section: Mutant Mice and Genotypingmentioning

confidence: 99%

Genetic rescue of Muenke syndrome model hearing loss reveals prolonged FGF-dependent plasticity in cochlear supporting cell fates

Mansour

Urness

2013

Genes Dev.

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“…Specific deletion of FGFR1 results in severe impairment of the development of hair cells and precursor cells 31 . FGF20 is specifically needed to differentiate the side constituents of the cochlea (including outer hair cells and Dieter's cells) 32 . In patients with low-frequency hearing, who undergo cochlear implantation, the maintenance of the remaining hair cells is essential.…”

Section: Discussionmentioning

confidence: 99%

In Vitro Differentiation of Human Bone Marrow Mesenchymal Stem Cells to Hair Cells Using Growth Factors

Mahmoudian-Sani¹,

Hashemzadeh‐Chaleshtori²,

Jami³

et al. 2017

The International Tinnitus Journal

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Objective: In this study, we attempted to differentiated human bone marrow-derived mesenchymal stem cells (hBMSCs) to auditory hair cells using growth factors. Methods: Retinoic acid (RA), basic fibroblast growth factor (bFGF), and epidermal growth factor (EGF) were added to hBMSCs cell culture medium. The cells were evaluated morphologically and the expression of SOX2, POU4F3, MYO7A, and Calretinin at mRNA level and ATOH1 mRNA and protein expression. Results: After treatment with the growth factors, the morphology of the cells did not change, but evaluation of gene expression at the mRNA level increased the expression of the ATOH1, SOX2, and POU4F3 markers. Growth factors increased the expression of ATOH1 at the protein level. The expression of calretinin showed decreased and MYO7A no significant change in expression. Conclusion: hBMSCs have the potential to differentiate to hair cell-like using the RA, bFGF, and EGF.

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“…Parallèlement, l'équipe de R. Kopan (Washington University, St Louis, États-Unis) avait généré une souris invalidée pour Fgf20 par insertion du gène de la -galactosidase (Gal) au niveau de l'exon 1. Outre des anomalies de diffé-renciation de la cochlée [10], les souris Fgf20 Gal/Gal développent des reins de taille réduite par rapport aux souris sauvages ( Figure 2B) [7]. Le croisement of the kidney and urinary tract) et constituent l'une des causes majeures d'insuffisance rénale chez l'enfant.…”

Section: Le Développement Rénalunclassified

Rôle crucial de FGF20 et FGF9 pour le maintien des progéniteurs rénaux lors du développement rénal

Jeanpierre

2013

Med Sci (Paris)

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Differentiation of the Lateral Compartment of the Cochlea Requires a Temporally Restricted FGF20 Signal

Abstract: FGF20 signaling in mice is required specifically for the differentiation of cochlear outer hair cells, the cells most often damaged during age-related hearing loss.

Cited by 100 publications

References 41 publications

Genetic rescue of Muenke syndrome model hearing loss reveals prolonged FGF-dependent plasticity in cochlear supporting cell fates

Genetic rescue of Muenke syndrome model hearing loss reveals prolonged FGF-dependent plasticity in cochlear supporting cell fates

In Vitro Differentiation of Human Bone Marrow Mesenchymal Stem Cells to Hair Cells Using Growth Factors

Rôle crucial de FGF20 et FGF9 pour le maintien des progéniteurs rénaux lors du développement rénal

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