The DOI matches its Crossref record; 21 of the 21 references it cites were found in a registry or an index.
“Everybody in the world is my friend” hypersociability in young children with Williams syndrome
Abstract
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. WS is caused by a hemizygous deletion in chromosome band 7q11.23, including the gene for elastin (ELN). Typically, individuals with WS seem driven to greet and interact with strangers. The goal of the present study was to investigate age‐related changes in the expression of hypersociability in WS. Parents of 64 children with WS, 31 children with Down syndrome (DS), and 27 normal controls (NC) provided data concerning specific aspects of their children's social behavior using the Salk Institute Sociability Questionnaire (SISQ). Children ranged in age from 1 year, 1 month to 12 years, 10 months. Consistent with earlier findings, whole group analyses showed the WS group to be significantly higher on all aspects of sociability studied. Comparisons among the groups at different ages revealed that hypersociability is evident even among very young children with WS, and, significantly, children with WS exceed children with DS with respect to Global Sociability and Approach Strangers in every age group. The findings from children who have the typical deletion for WS are contrasted with data obtained from a young child with WS who has a smaller deletion and many physical features of WS, but who does not demonstrate hypersociability, providing intriguing clues to a genetic basis of social behavior in this syndrome. These data suggest the involvement of a genetic predisposition in the expression of hypersociability in WS. © 2003 Wiley‐Liss, Inc.
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“There are currently three models of social function in WS: 1) heightened drive towards non-specific social interaction (for example, social interactions with strangers) [686,687], 2) heightened emotional responsiveness [685], and 3) social fearlessness [688].”
From Reward circuitry dysfunction in psychiatric and neurodevelopmental disorders and genetic syndromes: animal models and clinical findings (2012) · cites this paper as [686]
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Cites
Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome
Ewart, A. K., Morris, C. A., Atkinson, D., Jin, W., Sternes, K., Spallone, P., Stock, A. D., Leppert, M., & Keating, M. T. (1993) · Nature Genetics · doi:10.1038/ng0993-11 · Cited by 1,138
I. The Neurocognitive Profile of Williams Syndrome: A Complex Pattern of Strengths and Weaknesses
Bellugi, U., Lichtenberger, L., Jones, W., Lai, Z. C., & George, M. S. (2000) · Journal of Cognitive Neuroscience · doi:10.1162/089892900561959 · Cited by 706
Self-citationLIM-kinase1 Hemizygosity Implicated in Impaired Visuospatial Constructive Cognition
Frangiskakis, J. M., Ewart, A. K., Morris, C. A., Mervis, C. Β., Bertrand, J., Robinson, B. F., Klein, B. P., Ensing, G. J., Everett, L. A., Green, E. D., et al. (1996) · Cell · doi:10.1016/s0092-8674(00)80077-x · Cited by 555
II. Hypersociability in Williams Syndrome
Jones, W., Bellugi, U., Lai, Z., Chiles, M., Reilly, J., Lincoln, A., & Adolphs, R. (2000) · Journal of Cognitive Neuroscience · doi:10.1162/089892900561968 · Cited by 503
Self-citationBridging cognition, the brain and molecular genetics: evidence from Williams syndrome
Bellugi, U., Lichtenberger, L., Mills, D. L., Galaburda, A. M., & Korenberg, J. R. (1999) · Trends in Neurosciences · doi:10.1016/s0166-2236(99)01397-1 · Cited by 351
Self-citationWilliams syndrome: From genotype through to the cognitive phenotype
Donnai, D., & Karmiloff‐Smith, A. (2000) · American Journal of Medical Genetics · doi:10.1002/1096-8628(200022)97:2 · Cited by 314
Anxiety, Fears, and Phobias in Persons With Williams Syndrome
Dykens, E. M. (2003) · Developmental Neuropsychology · doi:10.1080/87565641.2003.9651896 · Cited by 289
VI. Genome structure and cognitive map of Williams syndrome
Korenberg, J. R., Chen, X.-N., Hirota, H., Lai, Z., Bellugi, U., Burian, D., Roe, B., & Matsuoka, R. (2000) · Journal of Cognitive Neuroscience · doi:10.1162/089892900562002 · Cited by 285
Self-citationElastin point mutations cause an obstructive vascular disease, supravalvular aortic stenosis
Li, D. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.7.1021 · Cited by 282
Williams Syndrome and Related Disorders
Morris, C. A., & Mervis, C. Β. (2000) · Annual Review of Genomics and Human Genetics · doi:10.1146/annurev.genom.1.1.461 · Cited by 247
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- Korenberg JR, 2001, Understanding the molecular genetic basis of human cognition: Evidence from Williams syndrome, Soc Neurosci (Abstract), 986, 5
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Automated apparatus for quantitation of social approach behaviors in mice
Nadler, J. J., Moy, S. S., Dold, G., Trang, D., Simmons, N., Perez, A., Young, N. B., Barbaro, R. P., Piven, J., Magnuson, T. R., et al. (2004) · Genes, Brain and Behavior · doi:10.1111/j.1601-183x.2004.00071.x · Cited by 775
Martens, M. A., Wilson, S. J., & Reutens, D. C. (2008) · Journal of Child Psychology and Psychiatry · doi:10.1111/j.1469-7610.2008.01887.x · Cited by 479
Language abilities in Williams syndrome: A critical review
Brock, J. (2007) · Development and Psychopathology · doi:10.1017/s095457940707006x · Cited by 335
Dichter, G. S., Damiano, C. A., & Allen, J. A. (2012) · Journal of Neurodevelopmental Disorders · doi:10.1186/1866-1955-4-19 · Cited by 299
Metadata
- Authors
- Doyle, T. F., Bellugi, U., Korenberg, J. R., & Graham, J.
- Affiliations
- Salk Institute for Biological Studies (US)
- Cedars-Sinai Medical Center (US)
- Corresponding author
- Doyle, Teresa F.
- Venue
- American Journal of Medical Genetics Part A
- Publisher
- Wiley
- Published
- 2003-08-13
- Volume
- 124A
- Issue
- 3
- Pages
- 263-273
- ISSN
- 1552-4825 (ISSN-L)
- 1552-4833
- OpenAlex
- W2080309563
- Cited by
- 296
- References
- 21
- Citations by year
- 2026: 7 · 2025: 5 · 2024: 13 · 2023: 5 · 2022: 8 · 2021: 16 · 2020: 9 · 2019: 7 · 2018: 12 · 2017: 14 · 2016: 20 · 2015: 18 · 2014: 16 · 2013: 20 · 2012: 14
- FWCI
- 2.04
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- American Journal of Medical Genetics Part A (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (1p01dc001289-01)
- National Institute on Deafness and Other Communication Disorders (P01 DC01289)
- National Institute of Neurological Disorders and Stroke (P50 NS 22343, P50NS022343)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (P01 HD 33113, P01HD033113)
- Topics
- Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
- Keywords
- Williams syndrome, hypersociability, social behavior, Down syndrome, age-related changes, chromosome 7q11.23 deletion, elastin gene
- MeSH terms
- Age Factors, Analysis of Variance, Child, Child, Preschool, Chromosome Deletion, Chromosomes, Human, Pair 7, Down Syndrome, Elastin, Family Health, Female, Genotype, Humans, Infant, Male, Surveys and Questionnaires, Social Behavior, Williams Syndrome
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