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The social phenotype of Williams syndrome

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CURRENT OPINION IN NEUROBIOLOGY
卷 23, 期 3, 页码 414-422

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CURRENT BIOLOGY LTD
DOI: 10.1016/j.conb.2012.12.006

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  1. NINDS [22343]
  2. Oak Tree Philanthropic Foundation
  3. [NICHD 033113-15]

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Williams syndrome (WS) offers an exciting model for social neuroscience because its genetic basis is well-defined, and the unique phenotype reflects dimensions of prosocial behaviors. WS is associated with a strong drive to approach strangers, a gregarious personality, heightened social engagement yet difficult peer interactions, high nonsocial anxiety, unusual bias toward positive affect, and diminished sensitivity to fear. New neurobiological evidence points toward alterations in structure, function, and connectivity of the social brain (amygdala, fusiform face area, orbital-frontal regions). Recent genetic studies implicate gene networks in the WS region with the dysregulation of prosocial neuropeptides. The study of WS has implications for understanding human social development, and may provide insight for translating genetic and neuroendocrine evidence into treatments for disorders of social behavior.

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