Expression of fragile X mental retardation protein within the vocal control system of developing and adult male zebra finches.
Expression of fragile X mental retardation protein within the vocal control system of developing and adult male zebra finches.
复制标题
发育中和成年雄性斑胸草雀声音控制系统中脆性 X 智力迟钝蛋白的表达。
DOI：
10.1016/j.neuroscience.2008.09.005
复制
发表时间：
2008
期刊：
影响因子：
3.3
通讯作者：
Ceman,S
中科院分区：
文献类型：
--
作者：
Winograd,C;Clayton,D;Ceman,S
患有脆性X综合征（FXS）的人认知受损，有明显的语言迟缓和缺陷。我们的目的是表征由Fmr1脆性X智力发育迟缓1基因或转录本（Fmr1）编码的脆性X智力发育迟缓蛋白（FMRP）在学习发声的动物模型，即斑胸草雀（Tgu）中的表达。我们克隆并测序了斑胸草雀FMR1的同源基因（TguFmr1），并开发了特异性识别TguFmrp的抗体。TguFmrp具有与其人类同源FMRP相似的结构特征。由于FXS患者表现出感觉运动缺陷，我们在斑马雀的感觉运动歌曲学习之前、期间和之后检测了TguFmrp的表达。我们发现TguFmrp在整个大脑和雄性斑胸草雀大脑的四个主要鸣叫核中表达，主要在神经元中表达。此外，在感觉运动学习之前，与周围的端脑相比，我们在孵化后第30天的雄性蝶节（RA）健壮核中观察到TguFmrp的表达升高，这表明这是为这一阶段的歌曲学习做准备。最后，我们观察到青少年和成年男性RA中TguFmrp表达的变化：在一些男性中，TguFmrp表达升高，而在另一些男性中，TguFmrp表达与周围的端脑相当。总之，我们描述了斑胸草雀FMRP的同源性，并发现运动前核RA在声音学习的关键发育阶段水平升高。
Individuals with fragile X syndrome (FXS) are cognitively impaired and have marked speech delays and deficits. Our goal was to characterize expression of fragile X mental retardation protein (FMRP), encoded by Fmr1 fragile X mental retardation 1 gene or transcript (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 robust nucleus of the arcopallium (RA) of post-hatch day 30 males, compared with 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.