Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome
Background. Waardenburg syndrome is an uncommon genetic condition characterized by at least some degree of congenital hearing loss and pigmentation deficiencies. However, the genetic pathway affecting the development of stria vascularis is not fully illustrated. Methods. The transcript profile of st...
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| Format: | Article |
| Language: | English |
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Wiley
2020-01-01
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| Series: | Neural Plasticity |
| Online Access: | http://dx.doi.org/10.1155/2020/2908182 |
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| author | Linjun Chen Lin Wang Lei Chen Fangyuan Wang Fei Ji Wei Sun Hui Zhao Weiju Han Shiming Yang |
| author_facet | Linjun Chen Lin Wang Lei Chen Fangyuan Wang Fei Ji Wei Sun Hui Zhao Weiju Han Shiming Yang |
| author_sort | Linjun Chen |
| collection | DOAJ |
| description | Background. Waardenburg syndrome is an uncommon genetic condition characterized by at least some degree of congenital hearing loss and pigmentation deficiencies. However, the genetic pathway affecting the development of stria vascularis is not fully illustrated. Methods. The transcript profile of stria vascularis of Waardenburg syndrome was studied using Mitf-M mutant pig and mice models. Therefore, GO analysis was performed to identify the differential gene expression caused by Mitf-M mutation. Results. There were 113 genes in tyrosine metabolism, melanin formation, and ion transportations showed significant changes in pig models and 191 genes in mice models. In addition, there were some spice’s specific gene changes in the stria vascularis in the mouse and porcine models. The expression of tight junction-associated genes, including Cadm1, Cldn11, Pcdh1, Pcdh19, and Cdh24 genes, were significantly higher in porcine models compared to mouse models. Vascular-related and ion channel-related genes in the stria vascularis were also shown significantly difference between the two species. The expression of Col2a1, Col3a1, Col11a1, and Col11a2 genes were higher, and the expression of Col8a2, Cd34, and Ncam genes were lower in the porcine models compared to mouse models. Conclusions. Our data suggests that there is a significant difference on the gene expression and function between these two models. |
| format | Article |
| id | doaj-art-b8b1561112474b55b01b7fd956066850 |
| institution | Kabale University |
| issn | 2090-5904 1687-5443 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Neural Plasticity |
| spelling | doaj-art-b8b1561112474b55b01b7fd9560668502025-02-03T01:04:00ZengWileyNeural Plasticity2090-59041687-54432020-01-01202010.1155/2020/29081822908182Transcript Profiles of Stria Vascularis in Models of Waardenburg SyndromeLinjun Chen0Lin Wang1Lei Chen2Fangyuan Wang3Fei Ji4Wei Sun5Hui Zhao6Weiju Han7Shiming Yang8College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, ChinaNursing Department, Hainan Hospital of Chinese PLA General Hospital, Sanya 572013, ChinaChongqing Academy of Animal Science, Chongqing 402460, ChinaCollege of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, ChinaCollege of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, ChinaDepartment of Communicative Disorders and Sciences, Center for Hearing and Deafness, The State University of New York at Buffalo, Buffalo, New York, USACollege of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, ChinaCollege of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, ChinaCollege of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, ChinaBackground. Waardenburg syndrome is an uncommon genetic condition characterized by at least some degree of congenital hearing loss and pigmentation deficiencies. However, the genetic pathway affecting the development of stria vascularis is not fully illustrated. Methods. The transcript profile of stria vascularis of Waardenburg syndrome was studied using Mitf-M mutant pig and mice models. Therefore, GO analysis was performed to identify the differential gene expression caused by Mitf-M mutation. Results. There were 113 genes in tyrosine metabolism, melanin formation, and ion transportations showed significant changes in pig models and 191 genes in mice models. In addition, there were some spice’s specific gene changes in the stria vascularis in the mouse and porcine models. The expression of tight junction-associated genes, including Cadm1, Cldn11, Pcdh1, Pcdh19, and Cdh24 genes, were significantly higher in porcine models compared to mouse models. Vascular-related and ion channel-related genes in the stria vascularis were also shown significantly difference between the two species. The expression of Col2a1, Col3a1, Col11a1, and Col11a2 genes were higher, and the expression of Col8a2, Cd34, and Ncam genes were lower in the porcine models compared to mouse models. Conclusions. Our data suggests that there is a significant difference on the gene expression and function between these two models.http://dx.doi.org/10.1155/2020/2908182 |
| spellingShingle | Linjun Chen Lin Wang Lei Chen Fangyuan Wang Fei Ji Wei Sun Hui Zhao Weiju Han Shiming Yang Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome Neural Plasticity |
| title | Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome |
| title_full | Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome |
| title_fullStr | Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome |
| title_full_unstemmed | Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome |
| title_short | Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome |
| title_sort | transcript profiles of stria vascularis in models of waardenburg syndrome |
| url | http://dx.doi.org/10.1155/2020/2908182 |
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