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Main Authors: Cheng, Yen-Fu, Kempfle, Judith, Chiang, Hao, Tani, Kohsuke, Wang, Quan, Chen, Sheng-Hong, Lenz, Danielle, Chen, Wei-Yi, Wu, Wenjin, Petrillo, Marco, Edge, Albert
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Published: Zenodo 2025
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Online Access:https://doi.org/10.5281/zenodo.14531704
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author Cheng, Yen-Fu
Kempfle, Judith
Chiang, Hao
Tani, Kohsuke
Wang, Quan
Chen, Sheng-Hong
Lenz, Danielle
Chen, Wei-Yi
Wu, Wenjin
Petrillo, Marco
Edge, Albert
author_facet Cheng, Yen-Fu
Kempfle, Judith
Chiang, Hao
Tani, Kohsuke
Wang, Quan
Chen, Sheng-Hong
Lenz, Danielle
Chen, Wei-Yi
Wu, Wenjin
Petrillo, Marco
Edge, Albert
contents <p>Stem cell pluripotency gene <em>Sox2</em> stimulates expression of proneural basic-helix-loop-helix transcription factor <em>Atoh1</em>. <em>Sox2</em> is necessary for the development of cochlear hair cells and binds to the <em>Atoh1</em> 3' enhancer to stimulate <em>Atoh1</em> expression. We show here that <em>Sox2</em> deletion in late embryogenesis results in the formation of extra hair cells, in contrast to the absence of hair cell development obtained after <em>Sox2</em> knockout early in gestation. <em>Sox2</em> overexpression decreased the level of Atoh1 protein despite an increase in <em>Atoh1</em> mRNA. Sox2 upregulated E3 ubiquitin ligase, <em>Huwe1</em>, by direct binding to the <em>Huwe1</em> gene. By upregulating its cognate E3 ligase, <em>Sox2</em> disrupts the positive feedback loop through which Atoh1 protein increases the expression of <em>Atoh1</em>. We conclude that <em>Sox2</em> initiates expression, while also limiting continued activity of bHLH transcription factor, Atoh1, and this inhibition represents a new mechanism for regulating the activity of this powerful initiator of hair cell development.</p>
format Recurso digital
id zenodo_https___doi_org_10_5281_zenodo_14531704
institution Zenodo
language
publishDate 2025
publisher Zenodo
record_format zenodo
spellingShingle Sox2 interacts with Atoh1 and Huwe1 loci to regulate Atoh1 transcription and stability during hair cell differentiation
Cheng, Yen-Fu
Kempfle, Judith
Chiang, Hao
Tani, Kohsuke
Wang, Quan
Chen, Sheng-Hong
Lenz, Danielle
Chen, Wei-Yi
Wu, Wenjin
Petrillo, Marco
Edge, Albert
Sox2
Huwe1
Atoh1
Cochlea
proteasome
<p>Stem cell pluripotency gene <em>Sox2</em> stimulates expression of proneural basic-helix-loop-helix transcription factor <em>Atoh1</em>. <em>Sox2</em> is necessary for the development of cochlear hair cells and binds to the <em>Atoh1</em> 3' enhancer to stimulate <em>Atoh1</em> expression. We show here that <em>Sox2</em> deletion in late embryogenesis results in the formation of extra hair cells, in contrast to the absence of hair cell development obtained after <em>Sox2</em> knockout early in gestation. <em>Sox2</em> overexpression decreased the level of Atoh1 protein despite an increase in <em>Atoh1</em> mRNA. Sox2 upregulated E3 ubiquitin ligase, <em>Huwe1</em>, by direct binding to the <em>Huwe1</em> gene. By upregulating its cognate E3 ligase, <em>Sox2</em> disrupts the positive feedback loop through which Atoh1 protein increases the expression of <em>Atoh1</em>. We conclude that <em>Sox2</em> initiates expression, while also limiting continued activity of bHLH transcription factor, Atoh1, and this inhibition represents a new mechanism for regulating the activity of this powerful initiator of hair cell development.</p>
title Sox2 interacts with Atoh1 and Huwe1 loci to regulate Atoh1 transcription and stability during hair cell differentiation
topic Sox2
Huwe1
Atoh1
Cochlea
proteasome
url https://doi.org/10.5281/zenodo.14531704