Shank3-deficient rats exhibit degraded cortical responses to sound
Shank3-deficient rats exhibit degraded cortical responses to sound
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DOI：
10.1002/aur.1883
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发表时间：
2018-01-01
期刊：
影响因子：
4.7
通讯作者：
Kilgard, Michael P.
中科院分区：
文献类型：
--
作者：
Engineer, Crystal T.;Rahebi, Kimiya C.;Kilgard, Michael P.
携带SHANK3基因突变的个体接受和表达语言的能力严重受损。虽然众所周知，这些人的大脑反应是异常的，但听觉皮质对声音的反应在很大程度上仍然没有得到充分的研究。在这项研究中，我们记录了新的Shank3基因缺陷大鼠模型中听觉皮质对语音和非语音的反应。我们预测，在Shank3基因缺陷的大鼠中，听觉皮质对声音的反应将受到损害。我们发现，与野生型大鼠相比，Shank3杂合子大鼠的听觉皮质反应更弱。此外，Shank3杂合子反应的自发听觉皮层放电较少，并且不能很好地应对快速的噪声爆发。SHANK3基因突变的大鼠听力障碍模型可用于测试潜在的康复或药物治疗，以改善Phelan-Mcdermid综合征患者观察到的沟通障碍。自闭症报告2018，11：59-68。(C)2017国际自闭症研究协会，Wiley Percials，Inc.发表摘要带有SHANK3突变的个体严重损害了语言能力，但听觉皮质对声音的反应在很大程度上仍未得到充分研究。在这项研究中，我们发现与对照组相比，Shank3基因缺陷大鼠的听觉皮质反应较弱，并且无法对快速声音做出很好的反应。SHANK3基因突变的大鼠听力障碍模型可用于测试潜在的康复或药物治疗，以改善Phelan-Mcdermid综合征患者观察到的沟通障碍。
Individuals with SHANK3 mutations have severely impaired receptive and expressive language abilities. While brain responses are known to be abnormal in these individuals, the auditory cortex response to sound has remained largely understudied. In this study, we document the auditory cortex response to speech and non-speech sounds in the novel Shank3-deficient rat model. We predicted that the auditory cortex response to sounds would be impaired in Shank3-deficient rats. We found that auditory cortex responses were weaker in Shank3 heterozygous rats compared to wild-type rats. Additionally, Shank3 heterozygous responses had less spontaneous auditory cortex firing and were unable to respond well to rapid trains of noise bursts. The rat model of the auditory impairments in SHANK3 mutation could be used to test potential rehabilitation or drug therapies to improve the communication impairments observed in individuals with Phelan-McDermid syndrome. Autism Res2018, 11: 59-68. (c) 2017 International Society for Autism Research, Wiley Periodicals, Inc.Lay SummaryIndividuals with SHANK3 mutations have severely impaired language abilities, yet the auditory cortex response to sound has remained largely understudied. In this study, we found that auditory cortex responses were weaker and were unable to respond well to rapid sounds in Shank3-deficient rats compared to control rats. The rat model of the auditory impairments in SHANK3 mutation could be used to test potential rehabilitation or drug therapies to improve the communication impairments observed in individuals with Phelan-McDermid syndrome.