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Sociability and synapse subtype-specific defects in mice lacking SRPX2, a language-associated gene.
PLOS ONE ( IF 2.9 ) Pub Date : 2018-06-19 , DOI: 10.1371/journal.pone.0199399 Breeanne M Soteros 1 , Qifei Cong 1 , Christian R Palmer 2 , Gek-Ming Sia 1
PLOS ONE ( IF 2.9 ) Pub Date : 2018-06-19 , DOI: 10.1371/journal.pone.0199399 Breeanne M Soteros 1 , Qifei Cong 1 , Christian R Palmer 2 , Gek-Ming Sia 1
Affiliation
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The FoxP2 transcription factor and its target genes have been implicated in developmental brain diseases with a prominent language component, such as developmental verbal dyspraxia and specific language impairment. How FoxP2 affects neural circuitry development remains poorly understood. The sushi domain protein SRPX2 is a target of FoxP2, and mutations in SRPX2 are associated with language defects in humans. We have previously shown that SRPX2 is a synaptogenic protein that increases excitatory synapse density. Here we provide the first characterization of mice lacking the SRPX2 gene, and show that these mice exhibit defects in both neural circuitry and communication and social behaviors. Specifically, we show that mice lacking SRPX2 show a specific reduction in excitatory VGlut2 synapses in the cerebral cortex, while VGlut1 and inhibitory synapses were largely unaffected. SRPX2 KO mice also exhibit an abnormal ultrasonic vocalization ontogenetic profile in neonatal pups, and reduced preference for social novelty. These data demonstrate a functional role for SRPX2 during brain development, and further implicate FoxP2 and its targets in regulating the development of vocalization and social circuits.
中文翻译：
缺少与语言相关的基因SRPX2的小鼠中的社交能力和突触亚型特异性缺陷。
FoxP2转录因子及其靶基因已与发展性脑疾病有关，具有突出的语言成分，例如发育性言语障碍和特定语言障碍。FoxP2如何影响神经回路发育仍然知之甚少。Sushi结构域蛋白SRPX2是FoxP2的靶标，SRPX2中的突变与人类的语言缺陷有关。先前我们已经表明SRPX2是一种可增加兴奋性突触密度的突触蛋白。在这里，我们提供了缺少SRPX2基因的小鼠的第一个特征，并显示这些小鼠在神经回路以及交流和社交行为方面均表现出缺陷。具体来说，我们显示缺少SRPX2的小鼠在大脑皮层中显示出兴奋性VGlut2突触的特异性减少，而VGlut1和抑制性突触在很大程度上不受影响。SRPX2 KO小鼠在新生幼仔中还表现出异常的超声发声个体遗传特征，并降低了对社交新颖性的偏好。这些数据证明了SRPX2在大脑发育过程中的功能作用，并进一步暗示了FoxP2及其靶标在调节发声和社交回路的发展中。
更新日期：2018-06-20
中文翻译：
缺少与语言相关的基因SRPX2的小鼠中的社交能力和突触亚型特异性缺陷。
FoxP2转录因子及其靶基因已与发展性脑疾病有关，具有突出的语言成分，例如发育性言语障碍和特定语言障碍。FoxP2如何影响神经回路发育仍然知之甚少。Sushi结构域蛋白SRPX2是FoxP2的靶标，SRPX2中的突变与人类的语言缺陷有关。先前我们已经表明SRPX2是一种可增加兴奋性突触密度的突触蛋白。在这里，我们提供了缺少SRPX2基因的小鼠的第一个特征，并显示这些小鼠在神经回路以及交流和社交行为方面均表现出缺陷。具体来说，我们显示缺少SRPX2的小鼠在大脑皮层中显示出兴奋性VGlut2突触的特异性减少，而VGlut1和抑制性突触在很大程度上不受影响。SRPX2 KO小鼠在新生幼仔中还表现出异常的超声发声个体遗传特征，并降低了对社交新颖性的偏好。这些数据证明了SRPX2在大脑发育过程中的功能作用，并进一步暗示了FoxP2及其靶标在调节发声和社交回路的发展中。
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