scholarly journals Diverse expression patterns of LIM-homeodomain transcription factors (LIM-HDs) in mammalian inner ear development

2008 ◽  
Vol 237 (11) ◽  
pp. 3305-3312 ◽  
Author(s):  
Mingqian Huang ◽  
Cyrille Sage ◽  
Huawei Li ◽  
Mengquig Xiang ◽  
Stefan Heller ◽  
...  
Keyword(s):  
Transcription Factors ◽  
Inner Ear ◽  
Expression Patterns ◽  
Ear Development ◽  
Inner Ear Development ◽  
Lim Homeodomain ◽  
Homeodomain Transcription Factors

Comparative analysis of conservation and regulatory network on core transcription factors in mouse inner ear development

2013 ◽  
Vol 35 (10) ◽  
pp. 1198-1208
Author(s):  
Zhi-Qiang CHEN ◽  
Xin-Huan HAN ◽  
Qin-Jun WEI ◽  
Guang-Qian XING ◽  
Xin CAO
Keyword(s):  
Transcription Factors ◽  
Comparative Analysis ◽  
Inner Ear ◽  
Regulatory Network ◽  
Ear Development ◽  
Inner Ear Development

scholarly journals 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

2021 ◽  
Vol 9 ◽  
Author(s):  
Jie Wen ◽  
Jian Song ◽  
Yijiang Bai ◽  
Yalan Liu ◽  
Xinzhang Cai ◽  
...  
Keyword(s):  
Gene Expression ◽  
Neural Crest ◽  
Inner Ear ◽  
Expression Patterns ◽  
Interaction Network ◽  
Patient Specific ◽  
Waardenburg Syndrome ◽  
Differentiation Potential ◽  
Ear Development ◽  
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 investigate the mechanisms of inherited human disease. In our work, we set up an iPSC line derived from a WS patient with SOX10 mutation and differentiated into neural crest cells (NCCs), a key cell type involved in inner ear development. Compared with control-derived iPSCs, the SOX10 mutant iPSCs showed significantly decreased efficiency of development and differentiation potential at the stage of NCCs. After that, we carried out high-throughput RNA-seq and evaluated the transcriptional misregulation at every stage. Transcriptome analysis of differentiated NCCs showed widespread gene expression alterations, and the differentially expressed genes (DEGs) were enriched in gene ontology terms of neuron migration, skeletal system development, and multicellular organism development, indicating that SOX10 has a pivotal part in the differentiation of NCCs. It’s worth noting that, a significant enrichment among the nominal DEGs for genes implicated in inner ear development was found, as well as several genes connected to the inner ear morphogenesis. Based on the protein-protein interaction network, we chose four candidate genes that could be regulated by SOX10 in inner ear development, namely, BMP2, LGR5, GBX2, and GATA3. In conclusion, SOX10 deficiency in this WS subject had a significant impact on the gene expression patterns throughout NCC development in the iPSC model. The DEGs most significantly enriched in inner ear development and morphogenesis may assist in identifying the underlying basis for the inner ear malformation in subjects with WS.

scholarly journals Residual microRNA expression dictates the extent of inner ear development in conditional Dicer knockout mice

2009 ◽  
Vol 328 (2) ◽  
pp. 328-341 ◽  
Author(s):  
Garrett A. Soukup ◽  
Bernd Fritzsch ◽  
Marsha L. Pierce ◽  
Michael D. Weston ◽  
Israt Jahan ◽  
...  
Keyword(s):  
Inner Ear ◽  
Knockout Mice ◽  
Microrna Expression ◽  
Ear Development ◽  
Inner Ear Development

scholarly journals Role of microRNAs in inner ear development and hearing loss

Gene ◽  
2019 ◽  
Vol 686 ◽  
pp. 49-55 ◽  
Author(s):  
Rahul Mittal ◽  
George Liu ◽  
Sai P. Polineni ◽  
Nicole Bencie ◽  
Denise Yan ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Ear Development ◽  
Inner Ear Development
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