PAX3 mutation suppress otic progenitors proliferation and induce apoptosis by inhibiting WNT1/beta-catenin signaling pathway in WS1 patient iPSC-derived inner ear organoids

pubmed: wnt1 2024-05-27

Summary:

Waardenburg syndrome type 1 (WS1) is a hereditary disease mainly characterized by sensorineural hearing loss, dystopia canthorum, and pigmentary defects. To elucidate molecular mechanisms underlying PAX3-associated hearing loss, we developed inner ear organoids model using induced pluripotent stem cells (iPSCs) derived from WS1 patient and healthy individual. Our results revealed a significant reduction in the size of inner ear organoids, accompanied by an increased level of apoptosis in...

Link:

https://pubmed.ncbi.nlm.nih.gov/38278051/?utm_source=Other&utm_medium=rss&utm_campaign=None&utm_content=16uwQpOeqFYN8R4TKOtwPy2utpqy9ex2oldalD2yF_fQHv2caq&fc=None&ff=20240527032055&v=2.18.0.post9+e462414

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Exome ยป pubmed: wnt1

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Authors:

SiJun Li, Chufeng He, Lingyun Mei, Xuewen Wu, Yong Feng, Jian Song

Date tagged:

05/27/2024, 03:21

Date published:

01/26/2024, 06:00