Exploring the zebra finch Taeniopygia guttata as a novel animal model for the speech-language deficit of fragile X syndrome.
Exploring the zebra finch Taeniopygia guttata as a novel animal model for the speech-language deficit of fragile X syndrome.
复制标题
DOI：
10.1007/978-3-642-21649-7_10
复制
发表时间：
2012
影响因子：
--
通讯作者：
Ceman, Stephanie
中科院分区：
文献类型：
--
作者：
Winograd, Claudia;Ceman, Stephanie
脆性 X 综合征 (FXS) 是遗传性智力障碍的最常见原因，并表现为明显非典型的言语，可能是由于声音学习受损所致。尽管当前的模型对于 FXS 某些方面的研究很有用，但斑胸草雀是唯一易于处理的声音学习实验室模型。斑胸草雀声音学习的神经回路与人脑中可能受到 FXS 影响的通路有明确的关系。此外，可以使用专门为此目的设计的软件来量化雀类的声音学习。斑胸草雀 FMR1 基因的敲除最终可能使新的针对具体治疗方式的测试成为可能，使用药物甚至社交策略来改善声音发育和功能的缺陷。在本章中，我们描述了斑胸草雀模型的实用性，并提出了 FMRP 在发声神经回路发育中的作用的假设。
Fragile X syndrome (FXS) is the most common cause of inherited intellectual disability and presents with markedly atypical speech-language, likely due to impaired vocal learning. Although current models have been useful for studies of some aspects of FXS, zebra finch is the only tractable lab model for vocal learning. The neural circuits for vocal learning in the zebra finch have clear relationships to the pathways in the human brain that may be affected in FXS. Further, finch vocal learning may be quantified using software designed specifically for this purpose. Knockdown of the zebra finch FMR1 gene may ultimately enable novel tests of therapies that are modality-specific, using drugs or even social strategies, to ameliorate deficits in vocal development and function. In this chapter, we describe the utility of the zebra finch model and present a hypothesis for the role of FMRP in the developing neural circuitry for vocalization.