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Expression of fragile X mental retardation protein within the vocal control system of developing and adult male zebra finches

机译:发育中和成年雄性斑马雀的声音控制系统中脆性X智力低下蛋白的表达

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摘要

Individuals with fragile X syndrome (FXS) are cognitively impaired and have marked speech delays and deficits. Our goal was to characterize expression of FMRP, the fragile X mental retardation protein, encoded by the gene FMR1, in an animal model that learns to vocalize, namely the zebra finch Taeniopygia guttata (Tgu). We cloned and sequenced the zebra finch ortholog of FMR1 (TguFmr1) and developed an antibody that recognizes TguFmrp specifically. TguFmrp has structural features similar to its human ortholog FMRP. Because FXS patients exhibit sensorimotor deficits, we examined TguFmrp expression prior to, during, and after sensorimotor song learning in zebra finches. We found that TguFmrp is expressed throughout the brain and in four major song nuclei of the male zebra finch brain, primarily in neurons. Additionally, prior to sensorimotor learning, we observed elevated TguFmrp expression in the RA of post-hatch day 30 males, compared to the surrounding telencephalon, suggesting a preparation for this stage of song learning. Finally, we observed variable TguFmrp expression in the RA of adolescent and adult males: in some males it was elevated and in others it was comparable to the surrounding telencephalon. In summary, we have characterized the zebra finch ortholog of FMRP and found elevated levels in the premotor nucleus RA at a key developmental stage for vocal learning.
机译:患有脆性X综合征(FXS)的个体在认知上受损,并且有明显的言语延迟和缺陷。我们的目标是鉴定在学习发声的动物模型即斑胸草雀雀鲷(Teniopygia guttata(Tgu))中由FMR1基因编码的脆弱的X智力低下的蛋白质FMRP的表达。我们克隆并测序了FMR1(TguFmr1)的斑马雀直向同源物,并开发了一种特异性识别TguFmrp的抗体。 TguFmrp具有类似于人类直系同源FMRP的结构特征。因为FXS患者表现出感觉运动缺陷,所以我们在斑马雀科感觉运动歌曲学习之前,期间和之后检查了TguFmrp表达。我们发现,TguFmrp在整个大脑以及雄性斑马雀科大脑的四个主要歌曲核中表达,主要在神经元中表达。另外,在感觉运动学习之前,我们观察到孵化后第30天男性的RA中TguFmrp表达升高,与周围的端脑相比,这表明该阶段的歌曲学习有所准备。最后,我们在青春期和成年男性的RA中观察到了可变的TguFmrp表达:在某些男性中,TguFmrp升高,而在其他男性中,其与周围的端脑相当。总而言之,我们已经对FMRP的斑雀雀直系同源物进行了表征,并在声乐学习的关键发展阶段发现了运动前核RA的水平升高。

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