Impaired geometric reorientation caused by genetic defect
Impaired geometric reorientation caused by genetic defect
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DOI：
10.1073/pnas.0909155107
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发表时间：
2010-02-16
影响因子：
11.1
通讯作者：
Landau, Barbara
中科院分区：
文献类型：
--
作者：
Lakusta, Laura;Dessalegn, Banchiamlack;Landau, Barbara
在环境中重新定位的能力是人类和非人类物种空间认知系统的基本部分。大量文献表明，人类成年人和幼儿、老鼠、雏鸟和鱼通过构建和使用周围布局的几何表示来完成重新定位，包括曲面的长度和它们的交点。这种重新定位系统的发展依赖于特定的基因及其在大脑发育中的作用吗？我们测试了患有威廉姆斯综合征(WS)的人的重定向，这是一种遗传性疾病，会导致已知参与重定向的大脑海马区和顶区的异常。我们发现，在缺乏表面特征信息的矩形洞室中，WS个体没有利用洞室的几何形状进行重定向，无法找到隐藏的对象。WS患者的失败不能用更普遍的视觉空间工作记忆缺陷来解释，因为同样的人在天花板上完成了类似的任务，他们没有迷失方向。我们还发现，在一个有一面蓝色墙壁的长方形房间里，WS患者的表现有所改善，这表明一些WS患者可以使用蓝色墙壁特征来定位隐藏的物体。这些结果表明，人类用于重新定位的几何系统可以被人类特定的遗传和神经异常选择性地破坏。
The capacity to reorient in one's environment is a fundamental part of the spatial cognitive systems of both humans and nonhuman species. Abundant literature has shown that human adults and toddlers, rats, chicks, and fish accomplish reorientation through the construction and use of geometric representations of surrounding layouts, including the lengths of surfaces and their intersection. Does the development of this reorientation system rely on specific genes and their action in brain development? We tested reorientation in individuals who have Williams syndrome (WS), a genetic disorder that results in abnormalities of hippocampal and parietal areas of the brain known to be involved in reorientation. We found that in a rectangular chamber devoid of surface feature information, WS individuals do not use the geometry of the chamber to reorient, failing to find a hidden object. The failure among people with WS cannot be explained by more general deficits in visual-spatial working memory, as the same individuals performed at ceiling in a similar task in which they were not disoriented. We also found that performance among people with WS improves in a rectangular chamber with one blue wall, suggesting that some individuals with WS can use the blue wall feature to locate the hidden object. These results show that the geometric system used for reorientation in humans can be selectively damaged by specific genetic and neural abnormalities in humans.