The role of Pendrin in the development of the murine inner ear

Wangemann P.

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The role of Pendrin in the development of the murine inner ear

机译：Pendrin在鼠内耳发育中的作用

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Enlargement of the vestibular aqueduct (EVA) is a common inner ear malformation found in children with sensorineural hearing loss that is frequently associated with loss-of-function or hypo-function mutations of SLC26A4. SLC26A4 codes for pendrin, which is a protein that is expressed in apical membranes of selected epithelia and functions as an anion exchanger. The comparatively high prevalence of EVA provides a strong imperative to develop rational interventions that delay, ameliorate or prevent hearing loss associated with this phenotype. The development of rational interventions requires a fundamental understanding of the role that pendrin plays in the normal development of hearing, as well as a detailed understanding of the pathobiologic mechanisms that, in the absence of fully functional pendrin, lead to an unstable hearing phenotype, with fluctuating or progressive loss of hearing. This review summarizes studies in mouse models that have focused on delineating the role of pendrin in the physiology of the inner ear and the pathobiology that leads to hearing loss.

机译：前庭导水管（EVA）的增大是在感音神经性听力减退的儿童中常见的内耳畸形，通常与SLC26A4的功能丧失或功能不足突变相关。 SLC26A4编码Pendrin，Pendrin是一种蛋白质，在选定的上皮的顶膜中表达，并充当阴离子交换剂。相对较高的EVA患病率是发展合理干预措施的强烈要求，这些干预措施可以延迟，改善或预防与该表型有关的听力损失。合理干预措施的发展需要对Pendrin在听力正常发育中所起的作用有基本的了解，并且需要详细了解在没有Pendrin的全部功能的情况下导致不稳定的听力表型的病理生物学机制。听力波动或渐进性丧失。这篇综述总结了在小鼠模型中的研究，这些研究着重于描述Pendrin在内耳生理和导致听力损失的病理生物学中的作用。

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《Cellular Physiology and Biochemistry》 |2011年第3期|共8页
作者
Wangemann P.;
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正文语种 eng
中图分类细胞生理学;
关键词
Cochlea; Development; Endolymphatic sac; Enlarged vestibular aqueduct; Genetic disease model; Hearing; Slc26a;

机译：耳蜗;发育;内淋巴囊;前庭导水管增大;遗传疾病模型;听力;Slc26a;

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1. The role of Pendrin in the development of the murine inner ear [J] . Wangemann P. Cellular Physiology and Biochemistry . 2011,第3期

机译：Pendrin在鼠内耳发育中的作用
2. Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear [J] . LORRAINE A. EVERETT, HAKIM MORSLI, DORIS K. WU Proceedings of the National Academy of Sciences of the United States of America . 1999,第17期

机译：Pendred综合征基因（Pds）的小鼠直系同源基因的表达模式表明Pendrin在内耳中起关键作用
3. CORRECTION: Surviving murine experimental sepsis affects the function and morphology of the inner ear CORRECTION: Surviving murine experimental sepsis affects the function and morphology of the inner ear CORRECTION: Surviving murine experimental sepsis affects the function and morphology of the inner ear [J] . Daniel Dejaco, Anneliese Schrott-Fischer, Nina Maria Mathonia, Biology Open . 2017,第9期

机译：更正：幸存的鼠实验性败血症影响内耳的功能和形态更正：幸存的鼠实验性败血症影响内耳的功能和形态更正：幸存的鼠实验性败血症影响内耳的功能和形态
4. New insights into the role of pendrin (SLC26A4) in inner ear fluid homeostasis [C] . Lorraine A. Everet Symposium on Epithelial anion transport in health and disease: the role of the SLC26 transporters family . 2006

机译：新的见解，进入脑膜内耳稳态中Pendrin（SLC26A4）的作用
5. Loss of Egr1 plays a role in murine leukemogenesis and may play a role in the development of acute myeloid leukemia and therapy-related acute myeloid leukemia. [D] . Joslin, John M. 2006

机译：Egr1的丢失在小鼠白血病的发生中起作用，并且可能在急性髓细胞性白血病和与治疗有关的急性髓细胞性白血病的发展中起作用。
6. Developmental Changes of ENaC Expression and Function in the Inner Ear of Pendrin Knock-Out Mice as a Perspective on the Development of Endolymphatic Hydrops [O] . Bo Gyung Kim, Jin Young Kim, Hee Nam Kim, -1

机译：从内淋巴积水发展的角度来看Pendrin基因敲除小鼠内耳ENaC表达和功能的发育变化。
7. Developmental changes of ENaC expression and function in the inner ear of pendrin knock-out mice as a perspective on the development of endolymphatic hydrops. [O] . Bo Gyung Kim, Jin Young Kim, Hee Nam Kim, 2014

机译：作为内淋巴水肿发展的观点，pendrin敲除小鼠内耳ENaC表达和功能的发育变化。

The role of Pendrin in the development of the murine inner ear

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