Structural variants in genes associated with human Williams-Beuren syndrome underlie stereotypical hypersociability in domestic dogs Bridgett Marie vonHoldt , Emily Shuldiner, Ilana Janowitz Koch, Rebecca Y. Kartzinel, Andrew Hogan, Lauren Brubaker, Shelby Wanser, Daniel Stahler, Clive D.L. Introduction. eCollection 2020. 30-46. However, the neural substrates of this hypersociability, i.e., positive attribution bias and increased drive toward social interaction, have not fully been elucidated. Porter MA , Coltheart M , Langdon R Neuropsychologia , 45(12):2839-2849, 21 May 2007 Prevention and treatment information (HHS). Codina-Sola M, Costa-Roger M, Pérez-García D, Flores R, Palacios-Verdú MG, Cusco I, Pérez-Jurado LA. Studies of abnormal populations provide a rare opportunity for examining relationships between cognition, genotype and brain neurobiology, permitting comparisons across these different levels of analysis. Linking cognitive neuroscience and molecular genetics: New Williams syndrome. Individuals with Williams syndrome (WS) demonstrate an abnormally positive social bias. People with Williams syndrome (WS) display indiscriminate approach toward strangers in everyday life. WS is caused by a hemizygous deletion in chromosome band … Privacy, Help https://resolver.caltech.edu/CaltechAUTHORS:JONjcn00. Hypersociability in Williams Syndrome. Search for other works by this author on: The Salk Institute for Biological Studies, California School of Professional Psychology. A new Tel Aviv University study finds that gene deletion or deficiency in neurons is responsible for the abnormal hypersocial behavior associated with Williams syndrome (WS), a rare disorder affecting 1 in 10,000 people around the world. Udell I. People with Williams syndrome (WS) display indiscriminate approach toward strangers in everyday life. Methods: We performed an event-related functional magnetic resonance imaging study while individuals with WS and … However, the neural substrates of this hypersociability, i.e., positive attribution bias and increased drive toward social interaction, have not fully been elucidated. 2020 Jan;50(1):127-144. doi: 10.1007/s10803-019-04232-5. Williams syndrome (WS), caused by a heterozygous microdeletion on chromosome 7q11.23, is a neurodevelopmental disorder characterized by hypersociability and neurocognitive abnormalities. People with Williams syndrome (WS) display indiscriminate approach toward strangers in everyday life. Hypersociability in Williams syndrome. Keywords: Williams syndrome, Hypersociability, Facial emotion, fMRI, Orbitofrontal cortex, Amygdala. They show strong predilection for using social evaluation in their stories, reflecting their hypersociability in language from an early age. This site needs JavaScript to work properly. Hypersociability in Williams syndrome result of myelination deficits Axon 'insulating tape' suffers from gene deletion that causes social … Careers. Williams-Beuren syndrome (WBS) is a developmental syndrome with unique neurocognitive features, including low IQ, deficits in visuospatial and visual-motor abilities, hypersensitivity to sounds, hypersociability, and increased general anxiety. 1999 May;22(5):197-207. doi: 10.1016/s0166-2236(99)01397-1. Trends Neurosci 5:197–208. J Cogn Neurosci 2000; 12 (Supplement 1): 30–46. Porter MA, Coltheart M, Langdon R. Neuropsychologia, 45(12):2839-2849, 21 May 2007 Cited by 112 articles | PMID: 17597166 Bellugi U, Lichtenberger L, Jones W, Lai Z, St George M. J Cogn Neurosci. a b c This page was last edited on 30 October 2018, at 12:42 (UTC). The syndrome affects about one in 10,000 people, and it is associated with a suite of mental and physical traits, including bubbly, extroverted personalities, a broad forehead, full cheeks, heart defects, intellectual disability and an affinity for music. Of the deleted genes, general transcription factor IIi (Gtf2i) has been linked to hypersociability in WS, although the underlying mechanisms are poorly understood. Neuroimaging studies conducted so far have linked this social profile to structural and/or functional abnormalities in WS amygdala and prefrontal cortex. regarding atypical, unfamiliar face processing in Williams syndrome (WS; Riby, Doherty-Sneddon, & Bruce, 2008a). Williams syndrome (WS) is a rare genetic disorder involving a characteristic cardiac defect, typical facial appearance, and an uneven profile of cognitive strengths and weaknesses. They show strong predilection for using social evaluation in their stories, reflecting their hypersociability in language from an early age. Four studies measuring distinct aspects of hypersocial behavior in WMS are presented, each probing specific aspects in WMS infants, toddlers, school age children, and adults. The team focused more specifically on 25 genes in this study known as Gtf2i and linked that gene to the hypersociability seen in Williams Syndrome. Towards the neural basis for hypersociability in a genetic syndrome. Abstract. Williams Syndrome, a rare neurodevelopmental disorder that affects about 1 in 10,000 babies born in the United States, produces a range of symptoms including cognitive impairments, cardiovascular problems, and extreme friendliness, or hypersociability. A critical component of such a cross-domain undertaking is the clear delineation of the phenotype of the disorder in question. Studies of abnormal populations provide a rare opportunity for examining relationships between cognition, genotype and brain neurobiology, permitting comparisons across these different levels of analysis. Am J Med Genet A. Hypersociability in Williams Syndrome In our studies, we investigate individuals with a rare, genetically based disorder called Williams syndrome (WMS) to draw links among these levels. In our studies, we investigate individuals with a rare, genetically based disorder called Williams syndrome (WMS) to draw links among these levels. A Behavioural Assessment of Social Anxiety and Social Motivation in Fragile X, Cornelia de Lange and Rubinstein-Taybi Syndromes. Hypersociability in Young Children With Williams Syndrome Teresa F. Doyle, 1 * Ursula Bellugi, 1 Julie R. Korenberg, 2,3 and John Graham 3 1 Laboratory forCognitive Neuroscience, The Salk Institute Biological Studies, La Jolla, California 2 University of California, Los Angeles, California 3 Cedars-Sinai Medical Center, Los Angeles, California However, the neural substrates of this hypersociability, i.e., positive attribution bias and increased drive toward social interaction, have not fully been 2004 Jan 30;124A(3):263-73. doi: 10.1002/ajmg.a.20416. Inappropriate approach behavior is also characteristic of people with acquired amygdala damage and people with acquired frontal lobe impairment; given this, the developmental disorder of … References. These studies of hypersocial behavior in WMS promise to provide the ground-work for crossdisciplinary analyses of gene-brain-behavior relationships. In our studies, we investigate individuals with a rare, genetically based disorder called Willi …. Trends Neurosci 5:197–208. National Library of Medicine Bellugi U, Adolphs R, Cassady C, Chiles M. Neuroreport. Williams syndrome signs and symptoms. However, the neural substrates of this hypersociability, i.e., positive attribution bias and increased drive toward social interaction, have not fully been elucidated. Furthermore, the studies show that the profile is observed across a wide range of ages, and emerges consistently across multiple experimental paradigms.
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