A Model of Waardenburg Syndrome Using Patient-Derived iPSCs With a SOX10 Mutation Displays Compromised Maturation and Function of the Neural Crest That Involves Inner Ear Development

A Model of Waardenburg Syndrome Using Patient-Derived iPSCs With a SOX10 Mutation Displays Compromised Maturation and Function of the Neural Crest That Involves Inner Ear Development

Waardenburg syndrome (WS) is an autosomal dominant inherited disorder that is characterized by sensorineural hearing loss and abnormal pigmentation. SOX10 is one of its main pathogenicity genes. The generation of patient-specific induced pluripotent stem cells (iPSCs) is an efficient means to invest...

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Bibliographic Details
Main Authors: Jie Wen, Jian Song, Yijiang Bai, Yalan Liu, Xinzhang Cai, Lingyun Mei, Lu Ma, Chufeng He, Yong Feng
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-08-01
Series:Frontiers in Cell and Developmental Biology
Subjects:
Waardenburg syndrome
SOX10
induced pluripotent stem cells (hiPSC)
neural crest cells (NCCs)
inner ear development
transcriptome analysis
Online Access:https://www.frontiersin.org/articles/10.3389/fcell.2021.720858/full

Internet

https://www.frontiersin.org/articles/10.3389/fcell.2021.720858/full

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