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迈向耳聋的基因治疗。

Towards gene therapy for deafness.

机构信息

Department of General Pathology, Second University of Naples, Naples, Italy.

出版信息

J Cell Physiol. 2011 Oct;226(10):2494-9. doi: 10.1002/jcp.22617.

DOI:10.1002/jcp.22617
PMID:21792906
Abstract

Many hearing disorders are associated with the damage or loss of sensory hair cells (HC) which can produce a profound and irreversible deafness. Apoptosis pathway is reported to play an important role leading to rapid expansion of the HC lesion after exposure to intense noise. Furthermore, progress made over the last year in understanding molecular mechanisms involved in the proliferative and regenerative capacity of sensory cells in the mammalian inner ear has raised the possibility that targeted therapies might prevent the loss of these cells and preserve the patient's hearing. A first step towards the successful therapeutic exploitation is a better understanding of the different pathways that control survival and proliferation of sensory cells. In this review, we provide an overview of recent findings concerning the possibility to prevent apoptosis in auditory cells. We also show the current knowledge on the molecular mechanisms involved in the potential regenerative behavior of these cells and the progress of gene therapy to prevent deafness noise-induced.

摘要

许多听力障碍与感觉毛细胞(HC)的损伤或丧失有关,而这可能导致严重且不可逆转的耳聋。有报道称,细胞凋亡途径在强噪声暴露后 HC 损伤迅速扩大中发挥重要作用。此外,过去一年在理解哺乳动物内耳感觉细胞增殖和再生能力相关的分子机制方面取得的进展,使得靶向治疗有可能防止这些细胞的丧失并维持患者的听力成为可能。成功进行治疗性开发的第一步是更好地了解控制感觉细胞存活和增殖的不同途径。在这篇综述中,我们概述了最近关于防止听觉细胞凋亡的可能性的发现。我们还展示了这些细胞潜在再生行为所涉及的分子机制的现有知识,以及基因治疗预防噪声诱导性耳聋的进展。

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High-throughput technologies for gene expression analyses: what we have learned for noise-induced cochlear degeneration?用于基因表达分析的高通量技术:我们从噪声性耳蜗退变中学到了什么?
J Otol. 2013 Jun;8(1):25-31. doi: 10.1016/S1672-2930(13)50003-1.
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Science. 2014 May 9;344(6184):1241062. doi: 10.1126/science.1241062.
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Transcriptome characterization by RNA-Seq reveals the involvement of the complement components in noise-traumatized rat cochleae.通过RNA测序进行转录组特征分析揭示补体成分参与噪声损伤的大鼠耳蜗。
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