• 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

纤毛在毛细胞发育、存活和再生中的作用。

The role of cilia in the development, survival, and regeneration of hair cells.

机构信息

Neuroscience Program, Lafayette College, Easton, PA 18042, USA.

Biology Department, Lafayette College, Easton, PA 18042, USA.

出版信息

Biol Open. 2024 Sep 15;13(9). doi: 10.1242/bio.061690. Epub 2024 Sep 12.

DOI:10.1242/bio.061690
PMID:39263863
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11413933/
Abstract

Mutations impacting cilia genes lead to a class of human diseases known as ciliopathies. This is due to the role of cilia in the development, survival, and regeneration of many cell types. We investigated the extent to which disrupting cilia impacted these processes in lateral line hair cells of zebrafish. We found that mutations in two intraflagellar transport (IFT) genes, ift88 and dync2h1, which lead to the loss of kinocilia, caused increased hair cell apoptosis. IFT gene mutants also have a decreased mitochondrial membrane potential, and blocking the mitochondrial uniporter causes a loss of hair cells in wild-type zebrafish but not mutants, suggesting mitochondria dysfunction may contribute to the apoptosis seen in these mutants. These mutants also showed decreased proliferation during hair cell regeneration but did not show consistent changes in support cell number or proliferation during hair cell development. These results show that the loss of hair cells seen following disruption of cilia through either mutations in anterograde or retrograde IFT genes appears to be due to impacts on hair cell survival but not necessarily development in the zebrafish lateral line.

摘要

影响纤毛基因的突变会导致一类被称为纤毛病的人类疾病。这是由于纤毛在许多细胞类型的发育、存活和再生中的作用。我们研究了破坏纤毛对斑马鱼侧线毛细胞这些过程的影响程度。我们发现，导致纤毛丧失的两个内鞭毛运输（IFT）基因 ift88 和 dync2h1 的突变导致毛细胞凋亡增加。IFT 基因突变体还具有降低的线粒体膜电位，并且阻断线粒体单向转运蛋白会导致野生型斑马鱼的毛细胞丧失，但突变体不会，这表明线粒体功能障碍可能导致这些突变体中观察到的细胞凋亡。这些突变体在毛细胞再生过程中也显示出增殖减少，但在毛细胞发育过程中支持细胞数量或增殖没有一致的变化。这些结果表明，通过正向或逆向 IFT 基因突变破坏纤毛后观察到的毛细胞丧失似乎是由于对毛细胞存活的影响，而不一定是斑马鱼侧线的发育。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/e15e02df9041/biolopen-13-061690-g8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/d698b91606d9/biolopen-13-061690-g1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/77adbac2e4b1/biolopen-13-061690-g2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/2a982f726fd7/biolopen-13-061690-g3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/60d4b9c8815a/biolopen-13-061690-g4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/ea9e3ab67d2d/biolopen-13-061690-g5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/1f6bffb92728/biolopen-13-061690-g6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/bf80235f2fd7/biolopen-13-061690-g7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/e15e02df9041/biolopen-13-061690-g8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/d698b91606d9/biolopen-13-061690-g1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/77adbac2e4b1/biolopen-13-061690-g2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/2a982f726fd7/biolopen-13-061690-g3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/60d4b9c8815a/biolopen-13-061690-g4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/ea9e3ab67d2d/biolopen-13-061690-g5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/1f6bffb92728/biolopen-13-061690-g6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/bf80235f2fd7/biolopen-13-061690-g7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df2a/11413933/e15e02df9041/biolopen-13-061690-g8.jpg

相似文献

1
The role of cilia in the development, survival, and regeneration of hair cells.纤毛在毛细胞发育、存活和再生中的作用。
Biol Open. 2024 Sep 15;13(9). doi: 10.1242/bio.061690. Epub 2024 Sep 12.
2
Cilia-Associated Genes Play Differing Roles in Aminoglycoside-Induced Hair Cell Death in Zebrafish.纤毛相关基因在斑马鱼氨基糖苷类诱导的毛细胞死亡中发挥不同作用。
G3 (Bethesda). 2016 Jul 7;6(7):2225-35. doi: 10.1534/g3.116.030080.
3
alms1 mutant zebrafish do not show hair cell phenotypes seen in other cilia mutants.alms1 突变斑马鱼没有表现出其他纤毛突变体中观察到的毛细胞表型。
PLoS One. 2021 Apr 1;16(4):e0246844. doi: 10.1371/journal.pone.0246844. eCollection 2021.
4
Loss of ift122, a Retrograde Intraflagellar Transport (IFT) Complex Component, Leads to Slow, Progressive Photoreceptor Degeneration Due to Inefficient Opsin Transport.IFT122（一种逆向鞭毛内运输（IFT）复合体成分）的缺失，由于视蛋白运输效率低下，导致缓慢、进行性的光感受器退化。
J Biol Chem. 2016 Nov 18;291(47):24465-24474. doi: 10.1074/jbc.M116.738658. Epub 2016 Sep 28.
5
foxg1a is required for hair cell development and regeneration in the zebrafish lateral line.foxg1a 在斑马鱼侧线毛细胞发育和再生中起作用。
Biol Open. 2024 Sep 15;13(9). doi: 10.1242/bio.060580. Epub 2024 Sep 20.
6
Phoenix is required for mechanosensory hair cell regeneration in the zebrafish lateral line.斑马鱼侧线机械感觉毛细胞再生需要Phoenix。
PLoS Genet. 2009 Apr;5(4):e1000455. doi: 10.1371/journal.pgen.1000455. Epub 2009 Apr 17.
7
The role of hair cells, cilia and ciliary motility in otolith formation in the zebrafish otic vesicle.毛细胞、纤毛和纤毛运动在斑马鱼耳泡耳石形成中的作用。
Development. 2012 May;139(10):1777-87. doi: 10.1242/dev.079947. Epub 2012 Mar 29.
8
Cilia-driven fluid flow as an epigenetic cue for otolith biomineralization on sensory hair cells of the inner ear.纤毛驱动的液流作为内耳感觉毛细胞内耳石生物矿化的表观遗传线索。
Development. 2011 Feb;138(3):487-94. doi: 10.1242/dev.057752.
9
Pregnancy-associated plasma protein-aa supports hair cell survival by regulating mitochondrial function.妊娠相关血浆蛋白 aa 通过调节线粒体功能支持毛细胞存活。
Elife. 2019 Jun 17;8:e47061. doi: 10.7554/eLife.47061.
10
Ciliary length regulation by intraflagellar transport in zebrafish.斑马鱼中鞭毛内运输对纤毛长度的调节
Elife. 2024 Dec 13;13:RP93168. doi: 10.7554/eLife.93168.

引用本文的文献

1
Primary cilia shape postnatal astrocyte development through Sonic Hedgehog signaling.初级纤毛通过音猬因子信号传导塑造出生后星形胶质细胞的发育。
J Cell Sci. 2025 Oct 15;138(20). doi: 10.1242/jcs.263965. Epub 2025 May 19.
2
Primary cilia shape postnatal astrocyte development through Sonic Hedgehog signaling.初级纤毛通过音猬因子信号传导塑造出生后星形胶质细胞的发育。
bioRxiv. 2024 Oct 17:2024.10.17.618851. doi: 10.1101/2024.10.17.618851.

本文引用的文献

1
Primary cilia support cartilage regeneration after injury.初级纤毛支持损伤后的软骨再生。
Int J Oral Sci. 2023 Jun 2;15(1):22. doi: 10.1038/s41368-023-00223-6.
2
Primary cilia as dynamic and diverse signalling hubs in development and disease.原发性纤毛作为发育和疾病中动态多样的信号枢纽。
Nat Rev Genet. 2023 Jul;24(7):421-441. doi: 10.1038/s41576-023-00587-9. Epub 2023 Apr 18.
3
Activity regulates a cell type-specific mitochondrial phenotype in zebrafish lateral line hair cells.活动调节斑马鱼侧线毛细胞中细胞类型特异性的线粒体表型。
Elife. 2023 Mar 13;12:e80468. doi: 10.7554/eLife.80468.
4
Primary cilia on muscle stem cells are critical to maintain regenerative capacity and are lost during aging.肌干细胞上的初级纤毛对于维持其再生能力至关重要，并且会随着衰老而丢失。
Nat Commun. 2022 Mar 17;13(1):1439. doi: 10.1038/s41467-022-29150-6.
5
Ciliary Hedgehog signaling regulates cell survival to build the facial midline.纤毛 Hedgehog 信号调节细胞存活以构建面部中线。
Elife. 2021 Oct 21;10:e68558. doi: 10.7554/eLife.68558.
6
Decreased IFT88 expression with primary cilia shortening causes mitochondrial dysfunction in cisplatin-induced tubular injury.IFT88 表达减少伴随初级纤毛缩短导致顺铂诱导的管状损伤中线粒体功能障碍。
Am J Physiol Renal Physiol. 2021 Sep 1;321(3):F278-F292. doi: 10.1152/ajprenal.00673.2020. Epub 2021 Aug 2.
7
Loss of vacuolar-type H+-ATPase induces caspase-independent necrosis-like death of hair cells in zebrafish neuromasts.空泡型质子泵的缺失诱导斑马鱼毛细胞发生胱天蛋白酶非依赖性的坏死样死亡。
Dis Model Mech. 2021 Jul 1;14(7). doi: 10.1242/dmm.048997. Epub 2021 Jul 23.
8
Loss of primary cilia promotes mitochondria-dependent apoptosis in thyroid cancer.原发性纤毛丢失促进甲状腺癌细胞中的线粒体依赖性凋亡。
Sci Rep. 2021 Feb 18;11(1):4181. doi: 10.1038/s41598-021-83418-3.
9
Kif3a deletion prevents primary cilia assembly on oligodendrocyte progenitor cells, reduces oligodendrogenesis and impairs fine motor function.Kif3a 缺失可阻止少突胶质前体细胞形成初级纤毛，减少少突胶质细胞生成并损害精细运动功能。
Glia. 2021 May;69(5):1184-1203. doi: 10.1002/glia.23957. Epub 2020 Dec 24.
10
Primary cilia mediate Klf2-dependant Notch activation in regenerating heart.初级纤毛介导 Klf2 依赖性 Notch 激活在再生心脏中。
Protein Cell. 2020 Jun;11(6):433-445. doi: 10.1007/s13238-020-00695-w. Epub 2020 Apr 5.