Comprehensive analysis of ultrasonic vocalizations in a mouse model of fragile X syndrome reveals limited, call type specific deficits.
Comprehensive analysis of ultrasonic vocalizations in a mouse model of fragile X syndrome reveals limited, call type specific deficits.
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DOI：
10.1371/journal.pone.0044816
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发表时间：
2012
期刊：
影响因子：
3.7
通讯作者：
Heck D
中科院分区：
文献类型：
--
作者：
Roy S;Watkins N;Heck D
脆性X综合征(FXS)是一种公认的遗传性智力低下，由脆性X智力低下1(Fmr1)基因突变引起。该基因位于X染色体的长臂上，编码脆性X智力低下蛋白(FMRP)。脆性X患者和Fmr1基因敲除(KO)小鼠中FMRP的缺失导致新皮质和海马区树突棘形成异常和突触可塑性改变等变化。FXS的临床特征包括认知障碍、焦虑、异常社交、智力低下、运动协调和言语清晰度障碍。当小鼠与母亲隔离时，会产生超声波发声(USV)。在FXS的小鼠模型中，这些社会超声发声是否存在缺陷尚不清楚。在这里，我们比较了由Fmr1-KO小鼠幼鼠产生的隔离诱导的USV与它们的野生型(WT)小鼠的幼鼠产生的USV。尽管Fmr1-KO小鼠的叫声总数在不同的基因型之间没有显著差异，但对10种不同类别的叫声的详细分析表明，Fmr1基因表达的缺失导致了超声波发声中有限的和叫声类型特异的缺陷：与它们的WT后代相比，Fmr1-KO小鼠的平坦叫声的载波频率更高，向下叫声的百分比更低，复杂叫声的频率范围更宽。
Fragile X syndrome (FXS) is a well-recognized form of inherited mental retardation, caused by a mutation in the fragile X mental retardation 1 (Fmr1) gene. The gene is located on the long arm of the X chromosome and encodes fragile X mental retardation protein (FMRP). Absence of FMRP in fragile X patients as well as in Fmr1 knockout (KO) mice results, among other changes, in abnormal dendritic spine formation and altered synaptic plasticity in the neocortex and hippocampus. Clinical features of FXS include cognitive impairment, anxiety, abnormal social interaction, mental retardation, motor coordination and speech articulation deficits. Mouse pups generate ultrasonic vocalizations (USVs) when isolated from their mothers. Whether those social ultrasonic vocalizations are deficient in mouse models of FXS is unknown. Here we compared isolation-induced USVs generated by pups of Fmr1-KO mice with those of their wild type (WT) littermates. Though the total number of calls was not significantly different between genotypes, a detailed analysis of 10 different categories of calls revealed that loss of Fmr1 expression in mice causes limited and call-type specific deficits in ultrasonic vocalization: the carrier frequency of flat calls was higher, the percentage of downward calls was lower and that the frequency range of complex calls was wider in Fmr1-KO mice compared to their WT littermates.
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