神经嵴对耳朵的贡献：对先天性听力障碍的影响。
Neural crest contributions to the ear: Implications for congenital hearing disorders.
机构信息
Department of Pediatrics, The University of Michigan Medical School, Ann Arbor, MI, USA.
Department of Pediatrics, The University of Michigan Medical School, Ann Arbor, MI, USA; Department of Human Genetics, University of Michigan Medical School, Ann Arbor, MI, USA.
出版信息
Hear Res. 2019 May;376:22-32. doi: 10.1016/j.heares.2018.11.005. Epub 2018 Nov 14.
Abstract
Congenital hearing disorders affect millions of children worldwide and can significantly impact acquisition of speech and language. Efforts to identify the developmental genetic etiologies of conductive and sensorineural hearing losses have revealed critical roles for cranial neural crest cells (NCCs) in ear development. Cranial NCCs contribute to all portions of the ear, and defects in neural crest development can lead to neurocristopathies associated with profound hearing loss. The molecular mechanisms governing the development of neural crest derivatives within the ear are partially understood, but many questions remain. In this review, we describe recent advancements in determining neural crest contributions to the ear, how they inform our understanding of neurocristopathies, and highlight new avenues for further research using bioinformatic approaches.
摘要
先天性听力障碍影响全球数以百万计的儿童，并可能严重影响言语和语言的习得。为了确定传导性和感觉神经性听力损失的发育遗传病因，研究人员揭示了颅神经嵴细胞（NCCs）在耳朵发育中的关键作用。颅神经嵴细胞为耳朵的所有部分提供贡献，而神经嵴发育缺陷可导致与严重听力损失相关的神经嵴病变。部分理解了控制耳朵中神经嵴衍生物发育的分子机制，但仍存在许多问题。在这篇综述中，我们描述了确定神经嵴对耳朵的贡献的最新进展，以及它们如何帮助我们理解神经嵴病变，并强调了使用生物信息学方法进一步研究的新途径。
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