Project 5: Characterization of Social Phenotype

威廉姆斯综合症（WS）最引人注目的特征之一是其独特的社会特征，这为理解为人类社会互动提供意义的潜在神经遗传系统提供了希望。到目前为止，我们的研究表明，虽然WS患者通常表现出增加的食欲社会驱动力，但其社会特征是以分离为特征的（例如，过度友好但交朋友困难；社交无所畏惧但焦虑；积极影响但适应不良行为）。项目V的目标集中在WS的社会表型特征，使这些“分离”的遗传和神经生物学途径联系起来。目标1：接近陌生人的贪得无厌的欲望驱动将研究WS患者对陌生人的吸引力和可接近性增加的基础和可变性。目的2：脸的独特显著性将阐明WS患者对脸的非典型高度兴趣的本质和潜在机制，以及它与由此产生的“超社会”表型的关系。目标3：不寻常的情绪敏感性会

One of the most compelling features of Williams syndrome (WS) is the distinctive social profile that holds promise for understanding the underlying neurogenetic systems that provide meaning to human social interaction. Our studies to date suggest that while individuals with WS typically demonstrate an increased appetitive social drive, the social profile is characterized by dissociations (e.g., overly-friendly with a difficulty in making friends; socially fearless but anxious; positive affect with maladaptive behaviors). The aims of Project V focus on the characterization of the social phenotype of WS, enabling links to the genetic and neurobiological pathways of these "dissociations". To this end, the Specific Aims are: Aim 1: Insatiable Appetitive Drive for Approaching Strangers will examine the underpinnings and variability of the increased attraction and approachability towards unfamiliar people observed in individuals with WS. Aim 2: The Unique Salience of Faces will elucidate the nature and underlying mechanisms of the atypically high interest in faces in WS, and its relation to the resultant "hypersocial" phenotype. Aim 3: Unusual Emotional Sensitivity will investigate both (a) the perception and processing of affect of others by those with WS, and (b) the overall affective style of individuals with WS. Using a multi-method design reflecting multiple levels of explanation (electrophysiology, autonomic function, and eye fixation). Project V studies will produce highly nuanced, quantifiable and independent key dimensions of the unique social behavior characteristic of WS. From a theoretical standpoint, a major thrust of the proposed work is to disentangle the processing of key components of social interaction and their respective underpinnings in the context of the enigmatic, yet paradoxical, WS social phenotype, with the ultimate goal of characterizing the complete system of social behavior and understanding the ways it can break down. Such a multileveled approach has not been previously adopted within this domain of inquiry. Results from these studies will add unique knowledge to our understanding of social behavior, by further defining the pathways implicated in gene-brain-behavior linkages, and are designed to contribute to better-informed treatments.