Arnett Megan T, Herman David H, McGee Aaron W
Developmental Neuroscience Program, Saban Research Institute, Children's Hospital Los Angeles, Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California, United States of America.
Section of Neurobiology, Department of Biological Sciences, University of Southern California, Los Angeles, California, United States of America.
PLoS One. 2014 Oct 8;9(10):e109116. doi: 10.1371/journal.pone.0109116. eCollection 2014.
The fragile X mental retardation 1 mutant mouse (Fmr1 KO) recapitulates several of the neurologic deficits associated with Fragile X syndrome (FXS). As tactile hypersensitivity is a hallmark of FXS, we examined the sensory representation of individual whiskers in somatosensory barrel cortex of Fmr1 KO and wild-type (WT) mice and compared their performance in a whisker-dependent learning paradigm, the gap cross assay. Fmr1 KO mice exhibited elevated responses to stimulation of individual whiskers as measured by optical imaging of intrinsic signals. In the gap cross task, initial performance of Fmr1 KO mice was indistinguishable from WT controls. However, while WT mice improved significantly with experience at all gap distances, Fmr1 KO mice displayed significant and specific deficits in improvement at longer distances which rely solely on tactile information from whiskers. Thus, Fmr1 KO mice possess altered cortical responses to sensory input that correlates with a deficit in tactile learning.
脆性X智力低下1突变小鼠(Fmr1基因敲除小鼠)再现了与脆性X综合征(FXS)相关的几种神经功能缺陷。由于触觉超敏是FXS的一个标志,我们检查了Fmr1基因敲除小鼠和野生型(WT)小鼠体感桶状皮层中单个 whisker 的感觉表征,并在依赖 whisker 的学习范式——间隙交叉试验中比较了它们的表现。通过内在信号的光学成像测量,Fmr1基因敲除小鼠对单个 whisker 的刺激表现出增强的反应。在间隙交叉任务中,Fmr1基因敲除小鼠的初始表现与野生型对照组没有区别。然而,虽然野生型小鼠在所有间隙距离下随着经验的增加都有显著改善,但Fmr1基因敲除小鼠在仅依赖 whisker 的触觉信息的较长距离改善方面表现出显著且特定的缺陷。因此,Fmr1基因敲除小鼠对感觉输入的皮层反应发生了改变,这与触觉学习缺陷相关。