Synergistic role of retinoic acid signaling and Gata3 during primitive choanae formation

Kurosaka, Hiroshi; Mushiake, Jin; Mithun, Saha; Wu, Yanran; Wang, Qi; Kikuchi, Masataka; Nakaya, Akihiro; Yamamoto, Sayuri; Inubushi, Toshihiro; Koga, Satoshi; Sandell, Lisa L.; Trainor, Paul; Yamashiro, Takashi

Synergistic role of retinoic acid signaling and Gata3 during primitive choanae formation

Kurosaka, Hiroshi; Mushiake, Jin; Mithun, Saha et al.

Number of Access:1,306（2025-08-14 05:02 Counts）

Identifier to cite or link to this item: https://hdl.handle.net/11094/79001

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Title	Synergistic role of retinoic acid signaling and Gata3 during primitive choanae formation
Creator	NRID 1000020509369 1000020509369 ORCID 0000-0002-8956-5467 0000-0002-8956-5467 researchmap permalink kurosakahiroshi kurosakahiroshi Scopus Author ID 11839162500 11839162500 CreatorName Kurosaka, Hiroshi
	CreatorName Mushiake, Jin
	CreatorName Mithun, Saha
	CreatorName Wu, Yanran
	CreatorName Wang, Qi
	NRID 1000090722538 1000090722538 CreatorName Kikuchi, Masataka
	NRID 1000060301149 1000060301149 CreatorName Nakaya, Akihiro
	CreatorName Yamamoto, Sayuri
	NRID 1000030550941 1000030550941 researchmap permalink opathone0141 opathone0141 Scopus Author ID 23097933700 23097933700 ORCID 0000-0001-5303-4763 0000-0001-5303-4763 CreatorName Inubushi, Toshihiro
	CreatorName Koga, Satoshi
	CreatorName Sandell, Lisa L.
	CreatorName Trainor, Paul
	NRID 1000070294428 1000070294428 ORCID 0000-0002-4419-9643 0000-0002-4419-9643 researchmap permalink read0051793 read0051793 Scopus Author ID 55416765600 55416765600 CreatorName Yamashiro, Takashi
Abstract	Developmental defects of primitive choanae, an anatomical path to connect the embryonic nasal and oral cavity, result in disorders called choanal atresia, which are associated with many congenital diseases and require immediate clinical intervention after birth. Previous studies revealed that reduced retinoid signaling underlies the etiology of choanal atresia. In the present study, by using multiple mouse models which conditionally deleted Rdh10 and Gata3 during embryogenesis, we showed that Gata3 expression is regulated by retinoid signaling during embryonic craniofacial development and plays crucial roles for development of the primitive choanae. Interestingly, Gata3 loss of function is known to cause hypoparathyroidism, sensorineural deafness and renal disease (HDR) syndrome, which exhibits choanal atresia as one of the phenotypes in humans. Our model partially phenocopies HDR syndrome with choanal atresia, and is thus a useful tool for investigating the molecular and cellular mechanisms of HDR syndrome. We further uncovered critical synergy of Gata3 and retinoid signaling during embryonic development, which will shed light on novel molecular and cellular etiology of congenital defects in primitive choanae formation.
Language	English
Handle URL	https://hdl.handle.net/11094/79001
Access Rights	open access
oaire:version	Author’s Original

Resource Type	other
Local Type	Preprint
DCMI Type	Text
DC.title	Synergistic role of retinoic acid signaling and Gata3 during primitive choanae formation
DC.creator	Kurosaka, Hiroshi
	Mushiake, Jin
	Mithun, Saha
	Wu, Yanran
	Wang, Qi
	Kikuchi, Masataka
	Nakaya, Akihiro
	Yamamoto, Sayuri
	Inubushi, Toshihiro
	Koga, Satoshi
	Sandell, Lisa L.
	Trainor, Paul
	Yamashiro, Takashi
DC.language' scheme='DCTERMS.RFC1766	English
DC.type' scheme='DCTERMS.DCMIType	Text
DCTERMS.abstract	Developmental defects of primitive choanae, an anatomical path to connect the embryonic nasal and oral cavity, result in disorders called choanal atresia, which are associated with many congenital diseases and require immediate clinical intervention after birth. Previous studies revealed that reduced retinoid signaling underlies the etiology of choanal atresia. In the present study, by using multiple mouse models which conditionally deleted Rdh10 and Gata3 during embryogenesis, we showed that Gata3 expression is regulated by retinoid signaling during embryonic craniofacial development and plays crucial roles for development of the primitive choanae. Interestingly, Gata3 loss of function is known to cause hypoparathyroidism, sensorineural deafness and renal disease (HDR) syndrome, which exhibits choanal atresia as one of the phenotypes in humans. Our model partially phenocopies HDR syndrome with choanal atresia, and is thus a useful tool for investigating the molecular and cellular mechanisms of HDR syndrome. We further uncovered critical synergy of Gata3 and retinoid signaling during embryonic development, which will shed light on novel molecular and cellular etiology of congenital defects in primitive choanae formation.
DC.format	79001
citation_title	Synergistic role of retinoic acid signaling and Gata3 during primitive choanae formation
citation_author	Kurosaka, Hiroshi
	Mushiake, Jin
	Mithun, Saha
	Wu, Yanran
	Wang, Qi
	Kikuchi, Masataka
	Nakaya, Akihiro
	Yamamoto, Sayuri
	Inubushi, Toshihiro
	Koga, Satoshi
	Sandell, Lisa L.
	Trainor, Paul
	Yamashiro, Takashi
citation_language	English
citation_public_url	https://hdl.handle.net/11094/79001

Synergistic role of retinoic acid signaling and Gata3 during primitive choanae formation

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