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Curr Opin Neurobiol. 2013 Jun;23(3):414-22. doi: 10.1016/j.conb.2012.12.006. Epub 2013 Jan 15.

The social phenotype of Williams syndrome.

Author information

1
Laboratory for Cognitive Neuroscience, the Salk Institute for Biological Studies, La Jolla, CA, USA.

Abstract

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.

PMID:
23332975
PMCID:
PMC4326252
DOI:
10.1016/j.conb.2012.12.006
[Indexed for MEDLINE]
Free PMC Article

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