The aim of the Program Project is to build cross-disciplinary research between cognition, brain and gene using Williams Syndrome (WMS) as a model. WMS is a rare genetically-based disorder that results in mental results in a characteristic cognitive profile of disssociations both within and across cognitive domains: (a) severe cognitive deficits but remarkably spared language, and (b) extreme disorders in spatial cognition, but preserved facial processing. These dissociations in higher cognitive functioning make WMS an invaluable paradigm for the study of brain and behavior relationships and the mapping of brain and behavior phenotypes to the genome.
Aim 1. Characterization and Expansion of the WMS Cognitive Phenotype. We will obtain standardized cognitive measures based on the profile of asymmetric cognitive functioning that characterizes WMS designed for cross level analyses.
Aim 2. Understanding the Processing Bases of Neurocognitive Dissociations in WMS. The major component of subproject 0001 involves experiments to investigate the basis of the unique cognitive architecture of WMS, organized along three themes, shared across all subprojects. A) Peaks and Valleys of Abilities in Visual Cognition. Experiments and in depth studies will examine the processing basis for the remarkably """"""""spared"""""""" face processing coupled with the extremely impaired spatial processing in WMS. b) Social Cognition. A social cognitive battery will characterize hypersociability and affect. c) Associations and Dissociations between Language and Other Domains. Studies will investigate the intersection of language and affect as well as the developing of language and spatial representations.
Aim 3. Mapping the Cognitive Phenotype to its Neural and Genetic Bases. Mapping across levels is coordinated with subprojects 0002-0005 working together interactively. Studies to date across projects suggests that the neural systems subserving cognitive functions in WMS are different from normal and have led us to a far more directed approach implicating the dorsal/ventral developmental pathways. By linking across projects and disciplines, we will begin to define features and pathways for the phenotypes of WMS. Summary. Studies of this genetically-based neurodevelopmental disorder will provide new opportunities to explore fundamental issues of cognitive neuroscience that relate cognitive functions to brain organization, as well as to their genetic bases.
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