• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

转录组分析揭示了 Pendred 综合征小鼠模型血管纹中 Hcy 代谢的改变。

Transcriptomic Analysis Reveals an Altered Hcy Metabolism in the Stria Vascularis of the Pendred Syndrome Mouse Model.

机构信息

Department of Otolaryngology-Head and Neck Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.

Otolaryngology Institute of Shanghai Jiao Tong University, Shanghai, China.

出版信息

Neural Plast. 2021 Apr 17;2021:5585394. doi: 10.1155/2021/5585394. eCollection 2021.

DOI:10.1155/2021/5585394
PMID:33959158
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8075705/
Abstract

PURPOSE

mice exhibit severer defects in the development of the cochlea and develop deafness, while the underlying mechanisms responsible for these effects remain unclear. Our study was to investigate the potential mechanism linking deficiency to hearing loss.

MATERIALS AND METHODS

RNA sequencing was applied to analyze the differential gene expression of the stria vascularis (SV) from wildtype and mice. GO and KEGG pathway analysis were performed. Quantitative RT-PCR was applied to validate the expression of candidate genes affected by . ELISA and immunofluorescence technique were used to detect the homocysteine (Hcy) level in serum, brain, and SV, respectively.

RESULTS

183 upregulated genes and 63 downregulated genes were identified in the SV associated with depletion. Transcriptomic profiling revealed that deficiency significantly affected the expression of genes associated with cell adhesion, transmembrane transport, and the biogenesis of multicellular organisms. The SV from mice exhibited a higher expression of mRNAs, as well as altered homocysteine (Hcy) metabolism.

CONCLUSIONS

The altered expression of results in a dramatic change in multiple biochemical reactions and a disruption of nutrient homeostasis in the endolymph which may contribute to hearing loss of knockout mouse.

摘要

目的

与野生型相比, 基因敲除小鼠耳蜗发育严重缺陷并致聋,但导致这些影响的潜在机制尚不清楚。本研究旨在探讨 基因缺失与听力损失之间的潜在联系。

材料和方法

采用 RNA 测序分析野生型和 基因敲除小鼠血管纹的差异基因表达。进行 GO 和 KEGG 通路分析。采用定量 RT-PCR 验证受 影响的候选基因的表达。采用 ELISA 和免疫荧光技术分别检测血清、脑组织和血管纹中的同型半胱氨酸(Hcy)水平。

结果

在与 基因耗竭相关的血管纹中,鉴定出 183 个上调基因和 63 个下调基因。转录组谱分析表明, 基因缺失显著影响与细胞黏附、跨膜转运和多细胞生物发生相关的基因表达。 基因敲除小鼠的血管纹中 mRNA 的表达增加,同型半胱氨酸(Hcy)代谢也发生改变。

结论

基因的异常表达导致多种生化反应的剧烈变化和内淋巴中营养物质稳态的破坏,这可能导致 基因敲除小鼠的听力损失。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/a0b3d8932f5b/NP2021-5585394.006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/e37deb5ce1ed/NP2021-5585394.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/68a0bd957bc6/NP2021-5585394.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/1b1a1b83ef41/NP2021-5585394.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/62d586d9a16e/NP2021-5585394.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/ff8c48b50407/NP2021-5585394.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/a0b3d8932f5b/NP2021-5585394.006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/e37deb5ce1ed/NP2021-5585394.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/68a0bd957bc6/NP2021-5585394.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/1b1a1b83ef41/NP2021-5585394.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/62d586d9a16e/NP2021-5585394.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/ff8c48b50407/NP2021-5585394.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a62d/8075705/a0b3d8932f5b/NP2021-5585394.006.jpg

相似文献

1
Transcriptomic Analysis Reveals an Altered Hcy Metabolism in the Stria Vascularis of the Pendred Syndrome Mouse Model.转录组分析揭示了 Pendred 综合征小鼠模型血管纹中 Hcy 代谢的改变。
Neural Plast. 2021 Apr 17;2021:5585394. doi: 10.1155/2021/5585394. eCollection 2021.
2
Single-cell RNA-sequencing of stria vascularis cells in the adult Slc26a4 mouse.成年 Slc26a4 小鼠血管纹细胞的单细胞 RNA 测序。
BMC Med Genomics. 2023 Jun 15;16(1):133. doi: 10.1186/s12920-023-01549-0.
3
Free radical stress-mediated loss of Kcnj10 protein expression in stria vascularis contributes to deafness in Pendred syndrome mouse model.血管纹中自由基应激介导的Kcnj10蛋白表达缺失导致彭德莱综合征小鼠模型耳聋。
Am J Physiol Renal Physiol. 2008 Jan;294(1):F139-48. doi: 10.1152/ajprenal.00433.2007. Epub 2007 Oct 24.
4
Macrophage invasion contributes to degeneration of stria vascularis in Pendred syndrome mouse model.巨噬细胞浸润导致 Pendred 综合征小鼠模型中血管纹的退变。
BMC Med. 2006 Dec 22;4:37. doi: 10.1186/1741-7015-4-37.
5
A knock-in mouse model of Pendred syndrome with Slc26a4 L236P mutation.携带 Slc26a4 L236P 突变的 Pendred 综合征敲入小鼠模型。
Biochem Biophys Res Commun. 2019 Jul 23;515(2):359-365. doi: 10.1016/j.bbrc.2019.05.157. Epub 2019 May 30.
6
Identification of common stria vascularis cellular alteration in sensorineural hearing loss based on ScRNA-seq.基于单细胞 RNA 测序的感音神经性听力损失中常见血管纹细胞改变的鉴定。
BMC Genomics. 2024 Feb 27;25(1):213. doi: 10.1186/s12864-024-10122-7.
7
Association of SLC26A4 mutations, morphology, and hearing in pendred syndrome and NSEVA.Pendred 综合征和 NSEVA 中 SLC26A4 突变、形态和听力的相关性。
Laryngoscope. 2019 Nov;129(11):2574-2579. doi: 10.1002/lary.27319.
8
Gene therapy for hereditary hearing loss by SLC26A4 mutations in mice reveals distinct functional roles of pendrin in normal hearing.基因治疗小鼠 SLC26A4 突变所致遗传性听力损失揭示了 Pendrin 在正常听力中的不同功能作用。
Theranostics. 2019 Sep 23;9(24):7184-7199. doi: 10.7150/thno.38032. eCollection 2019.
9
Connexin30 deficiency causes instrastrial fluid-blood barrier disruption within the cochlear stria vascularis.连接蛋白30缺乏会导致耳蜗血管纹内的内淋巴液-血液屏障破坏。
Proc Natl Acad Sci U S A. 2007 Apr 10;104(15):6229-34. doi: 10.1073/pnas.0605108104. Epub 2007 Mar 30.
10
Mutation analysis of SLC26A4 (Pendrin) gene in a Brazilian sample of hearing-impaired subjects.巴西听力受损受试者样本中SLC26A4(Pendrin)基因的突变分析。
BMC Med Genet. 2018 May 8;19(1):73. doi: 10.1186/s12881-018-0585-x.

引用本文的文献

1
The role of the stria vascularis in neglected otologic disease.血管纹在被忽视的耳科学疾病中的作用。
Hear Res. 2023 Feb;428:108682. doi: 10.1016/j.heares.2022.108682. Epub 2022 Dec 24.
2
Role of the Stria Vascularis in the Pathogenesis of Sensorineural Hearing Loss: A Narrative Review.血管纹在感音神经性听力损失发病机制中的作用:一项叙述性综述。
Front Neurosci. 2021 Nov 19;15:774585. doi: 10.3389/fnins.2021.774585. eCollection 2021.

本文引用的文献

1
2D TiCTMXene couples electrical stimulation to promote proliferation and neural differentiation of neural stem cells.二维 TiCTMXene 通过电刺激促进神经干细胞的增殖和神经分化。
Acta Biomater. 2022 Feb;139:105-117. doi: 10.1016/j.actbio.2020.12.035. Epub 2020 Dec 31.
2
Disruption of Atg7-dependent autophagy causes electromotility disturbances, outer hair cell loss, and deafness in mice.Atg7 依赖性自噬的破坏导致小鼠的运动电活动障碍、外毛细胞丧失和耳聋。
Cell Death Dis. 2020 Oct 24;11(10):913. doi: 10.1038/s41419-020-03110-8.
3
The gene is required for hair cell development in zebrafish.
该基因对于斑马鱼毛细胞的发育是必需的。
Aging (Albany NY). 2020 Oct 12;12(19):18804-18821. doi: 10.18632/aging.103840.
4
Transcriptomic analysis highlights cochlear inflammation associated with age-related hearing loss in C57BL/6 mice using next generation sequencing.转录组分析通过新一代测序突出了C57BL/6小鼠中与年龄相关性听力损失相关的耳蜗炎症。
PeerJ. 2020 Aug 19;8:e9737. doi: 10.7717/peerj.9737. eCollection 2020.
5
Gene therapy development in hearing research in China.中国听力研究中的基因治疗发展。
Gene Ther. 2020 Aug;27(7-8):349-359. doi: 10.1038/s41434-020-0177-1. Epub 2020 Jul 17.
6
Blebbistatin Inhibits Neomycin-Induced Apoptosis in Hair Cell-Like HEI-OC-1 Cells and in Cochlear Hair Cells.blebbistatin抑制新霉素诱导的类毛细胞HEI-OC-1细胞和耳蜗毛细胞凋亡。
Front Cell Neurosci. 2020 Feb 5;13:590. doi: 10.3389/fncel.2019.00590. eCollection 2019.
7
Three-Dimensional Graphene Enhances Neural Stem Cell Proliferation Through Metabolic Regulation.三维石墨烯通过代谢调控增强神经干细胞增殖。
Front Bioeng Biotechnol. 2020 Jan 8;7:436. doi: 10.3389/fbioe.2019.00436. eCollection 2019.
8
Age-related transcriptome changes in Sox2+ supporting cells in the mouse cochlea.年龄相关的 Sox2+ 支持细胞在小鼠耳蜗中的转录组变化。
Stem Cell Res Ther. 2019 Dec 2;10(1):365. doi: 10.1186/s13287-019-1437-0.
9
Pre-treatment With Fasudil Prevents Neomycin-Induced Hair Cell Damage by Reducing the Accumulation of Reactive Oxygen Species.法舒地尔预处理通过减少活性氧的积累预防新霉素诱导的毛细胞损伤。
Front Mol Neurosci. 2019 Nov 6;12:264. doi: 10.3389/fnmol.2019.00264. eCollection 2019.
10
The nuclear transcription factor FoxG1 affects the sensitivity of mimetic aging hair cells to inflammation by regulating autophagy pathways.核转录因子 FoxG1 通过调节自噬途径影响模拟衰老毛细胞对炎症的敏感性。
Redox Biol. 2020 Jan;28:101364. doi: 10.1016/j.redox.2019.101364. Epub 2019 Oct 29.