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增强型S锥体综合征:S锥体数量异常增多的证据。

Enhanced S cone syndrome: evidence for an abnormally large number of S cones.

作者信息

Hood D C, Cideciyan A V, Roman A J, Jacobson S G

机构信息

Department of Psychology, Columbia University, New York, NY 10027, USA.

出版信息

Vision Res. 1995 May;35(10):1473-81. doi: 10.1016/0042-6989(95)98727-q.

DOI:10.1016/0042-6989(95)98727-q
PMID:7645276
Abstract

The cellular basis of the hypersensitivity of the S (blue) cone system in patients with enhanced S cone syndrome was examined by analyzing ERGs from three patients. The patients had large alpha-waves in response to the blue and white flashes. These alpha-waves were shown to be driven nearly entirely by the S cones. Although these S cone alpha-waves were 4-6 times the size of the normal L/M cone alpha-wave, they are of the same form, and could be quantitatively described with the same model previously shown to fit cone alpha-waves. We propose that the retina of these patients has many more S cones than the normal retina and that these cones replace some of the normal L/M cones and many of the rods.

摘要

通过分析三名增强型S视锥综合征患者的视网膜电图(ERG),研究了该疾病患者S(蓝色)视锥系统超敏反应的细胞基础。这些患者对蓝色和白色闪光的反应有较大的α波。结果显示,这些α波几乎完全由S视锥驱动。尽管这些S视锥α波的大小是正常L/M视锥α波的4至6倍,但它们的波形相同,并且可以用先前显示适用于视锥α波的相同模型进行定量描述。我们提出,这些患者的视网膜中S视锥的数量比正常视网膜多得多,并且这些视锥取代了一些正常的L/M视锥和许多视杆细胞。

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