A naturally occurring mouse model of X-linked congenital stationary night blindness.
A naturally occurring mouse model of X-linked congenital stationary night blindness.
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DOI：
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发表时间：
1998-11
影响因子：
4.4
通讯作者：
M. Pardue;M. McCall;M. Lavail;R. Gregg;N. Peachey
中科院分区：
文献类型：
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作者：
M. Pardue;M. McCall;M. Lavail;R. Gregg;N. Peachey
目的描述一种自然发生的X连锁隐性突变--无b波(Nob)，它损害了小鼠光感受器和二级神经元之间的视觉传递。方法通过记录小鼠视网膜光诱发反应、视网膜电信号(ERG)进行识别。为了评估视觉传递，用头皮电极记录了皮层电位。NOB的遗传模式是通过用正常小鼠饲养NOB动物来确定的。光镜下进行视网膜组织学分析。结果NOB小鼠虽然感光细胞介导的ERG成分(a波)正常，但反映受体后神经元活动的主要反应成分(b波)缺失。视觉驱动的皮质活动在nob动物中也是异常的。在光镜水平上，头部视网膜似乎具有正常的细胞结构。结论小鼠视网膜头部缺陷干扰了视觉信息的传递，是研究视网膜外突触功能的有用模型。此外，这个突变的小鼠似乎为完全性先天性静止性夜盲提供了一个动物模型，这是一种人类疾病，患者严重丧失了杆介导的视觉敏感度。
PURPOSE To describe a naturally occurring X-linked recessive mutation, no b-wave (nob), that compromises visual transmission between photoreceptors and second-order neurons in mice. METHODS Affected mice were identified by recording the light-evoked response of the retina, the electroretinogram (ERG). To evaluate visual transmission, cortical potentials were recorded with a scalp electrode. The inheritance pattern for nob was defined by breeding nob animals with normal mice. Retinal histologic analysis was performed by light microscopy. RESULTS Although the photoreceptor-mediated ERG component (a-wave) was normal in nob mice, the major response component reflecting postreceptoral neuronal activity (b-wave) was missing. Visually-driven cortical activity was also abnormal in nob animals. At the light microscopic level, the nob retina appeared to have a normal cytoarchitecture. CONCLUSIONS These findings suggest that the nob defect interferes with the transmission of visual information through the retina and that these mice are a useful model for the study of outer retinal synaptic function. In addition, this mutant mouse seems to provide an animal model for the complete form of congenital stationary night blindness, a human disorder in which patients have a profound loss of rod-mediated visual sensitivity.