The cochlea is built to last a lifetime.
The cochlea is built to last a lifetime.
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DOI：
10.1016/j.heares.2023.108821
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发表时间：
2023-09-01
期刊：
影响因子：
2.8
通讯作者：
Savas, Jeffrey N.
中科院分区：
文献类型：
--
作者：
Savas, Jeffrey N.
关键词：
蛋白质的产生和降解以及蛋白质寿命的调节在许多基本生物过程中起着核心作用。几乎所有的哺乳动物蛋白质都是通过蛋白质的合成和降解来补充的。蛋白质在体内的寿命通常以天为单位测量，但少数极长寿命蛋白质（ELLP）持续数月甚至数年。ELLP在所有组织中都是罕见的，但在含有终末分化的有丝分裂后细胞和细胞外基质的组织中富集。一致的，新出现的证据表明，耳蜗可能是特别丰富的ELLP。对特殊细胞类型中的ELLP的损伤，例如眼睛的透镜细胞中的晶状体蛋白，导致器官衰竭，例如白内障。类似地，耳蜗ELLP的损伤可能发生在许多损伤中，包括声学过度刺激、药物、缺氧和抗生素，并且可能在听力损失中发挥未被充分认识的作用。此外，蛋白质降解受阻可能导致后天性听力损失。在这篇综述中，我强调我们的知识的耳蜗蛋白的寿命，重点是ELLP和潜在的贡献，受损的耳蜗蛋白降解获得性听力损失和新兴的相关性ELLP。
Orchestration of protein production and degradation and the regulation of protein lifetimes play a central role in many basic biological processes. Nearly all mammalian proteins are replenished by protein turnover in waves of synthesis and degradation. Protein lifetimes in vivo are typically measured in days, but a small number of extremely long-lived proteins (ELLPs) persist for months or even years. ELLPs are rare in all tissues but are enriched in tissues containing terminally differentiated post-mitotic cells and extracellular matrix. Consistently, emerging evidence suggests that the cochlea may be particularly enriched in ELLPs. Damage to ELLPs in specialized cell types, such as crystallin in the lens cells of the eye, causes organ failure such as cataracts. Similarly, damage to cochlear ELLPs is likely to occur with many insults, including acoustic overstimulation, drugs, anoxia, and antibiotics, and may play an underappreciated role in hearing loss. Furthermore, hampered protein degradation may contribute to acquired hearing loss. In this review, I highlight our knowledge of the lifetimes of cochlear proteins with an emphasis on ELLPs and the potential contribution that impaired cochlear protein degradation has on acquired hearing loss and the emerging relevance of ELLPs.
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DOI：
10.1073/pnas.1821844116
发表时间：
2019-04-16
影响因子：
11.1
作者：
Defourny, Jean;Aghaie, Alain;Petit, Christine
通讯作者：
Petit, Christine
影响因子：
4.7
作者：
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影响因子：
5.3
作者：
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通讯作者：
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DOI：
10.1073/pnas.94.26.14450
发表时间：
1997-12-23
影响因子：
11.1
作者：
Cohen-Salmon, M;El-Amraoui, A;Petit, C
通讯作者：
Petit, C
影响因子：
16.6
作者：
Fornasiero EF;Mandad S;Wildhagen H;Alevra M;Rammner B;Keihani S;Opazo F;Urban I;Ischebeck T;Sakib MS;Fard MK;Kirli K;Centeno TP;Vidal RO;Rahman RU;Benito E;Fischer A;Dennerlein S;Rehling P;Feussner I;Bonn S;Simons M;Urlaub H;Rizzoli SO
通讯作者：
Rizzoli SO