Neural mechanisms in Williams syndrome: a unique window to genetic influences on cognition and behaviour

No abstract is available for this paper.

• Publication year

  2006

• Venue

  Nature Reviews Neuroscience

• Publication date

  2006-05-01

• Fields of study

  Biology, Medicine, Psychology

• Identifiers
  DOI 10.1038/nrn1906PMID 16760918
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Neural mechanisms of genetic risk for impulsivity and violence in humans.

  2006cited by this paper
• Perceiving facial and vocal expressions of emotion in individuals with Williams syndrome.

  2006cited by this paper
• Prevalence of psychiatric disorders in 4 to 16‐year‐olds with Williams syndrome

  2006cited by this paper
• Williams-Beuren syndrome : research, evaluation, and treatment

  2006cited by this paper
• Parieto-occipital grey matter abnormalities in children with Williams syndrome

  2006cited by this paper
• Is everybody always my friend? Perception of approachability in Williams syndrome.

  2006cited by this paper
• Review and Future Directions

  2006cited by this paper
• Object recognition with severe spatial deficits in Williams syndrome: sparing and breakdown.

  2006cited by this paper
• Neuroanatomic observations of the brain in autism: a review and future directions

  2005cited by this paper
• Severe expressive-language delay related to duplication of the Williams-Beuren locus.

  2005cited by this paper
• Genetic Contributions to Human Gyrification: Sulcal Morphometry in Williams Syndrome

  2005cited by this paper
• Autism: a window onto the development of the social and the analytic brain.

  2005cited by this paper
• Music and anxiety in Williams syndrome: a harmonious or discordant relationship?

  2005cited by this paper
• An Event-Related Functional Magnetic Resonance Imaging Study of Voluntary and Stimulus-Driven Orienting of Attention

  2005cited by this paper
• Neuropsychological, neurological, and neuroanatomical profile of Williams syndrome.

  2005cited by this paper
• Abnormal Cortical Complexity and Thickness Profiles Mapped in Williams Syndrome

  2005cited by this paper
• Multiple Object Tracking in People With Williams Syndrome and in Normally Developing Children

  2005cited by this paper
• Foreshortened dorsal extension of the central sulcus in Williams syndrome.

  2005cited by this paper
• Knowledge of constraints on compounding in children and adolescents with Williams syndrome.

  2005cited by this paper
• Sensorineural hearing loss in children and adults with Williams syndrome

  2005cited by this paper
• GTF2IRD1 in Craniofacial Development of Humans and Mice

  2005cited by this paper
• Functional, structural, and metabolic abnormalities of the hippocampal formation in Williams syndrome.

  2005cited by this paper
• Williams syndrome: pediatric, neurologic, and cognitive development.

  2005cited by this paper
• Gaze fixation and the neural circuitry of face processing in autism

  2005cited by this paper
• Expressive language of children with Williams syndrome.

  2005cited by this paper
• Evidence for superior parietal impairment in Williams syndrome

  2005cited by this paper
• Visual and spatial long‐term memory: differential pattern of impairments in Williams and Down syndromes

  2005cited by this paper
• Neural correlates of genetically abnormal social cognition in Williams syndrome

  2005cited by this paper
• Interdependence of N‐acetyl aspartate and high‐energy phosphates in healthy human brain

  2005cited by this paper
• Hippocampal and visuospatial learning defects in mice with a deletion of frizzled 9, a gene in the Williams syndrome deletion interval

  2005cited by this paper
• The Development of Social Behavior Following Neonatal Amygdala Lesions in Rhesus Monkeys

  2004cited by this paper
• Pragmatic language impairment and social deficits in Williams syndrome: a comparison with Down's syndrome and specific language impairment.

  2004cited by this paper
• Neural correlates of facial affect processing in children and adolescents with autism spectrum disorder.

  2004cited by this paper
• Genetic Control of Cortical Convolutions

  2004cited by this paper
• Neuroanatomical substrates of social cognition dysfunction in autism.

  2004cited by this paper
• Mechanisms of verbal memory impairment in four neurodevelopmental disorders.

  2004cited by this paper
• Neuroscience. Genetic control of cortical convolutions.

  2004cited by this paper
• Distinct developmental trajectories in three systems for spatial encoding between the ages of 3 and 6 years

  2004cited by this paper
• Multisystem study of 20 older adults with Williams syndrome

  2004cited by this paper
• Hippocampal contribution to the novel use of relational information in declarative memory

  2004cited by this paper
• Characterizing the Musical Phenotype in Individuals With Williams Syndrome

  2004cited by this paper
• An Experiment of Nature: Brain Anatomy Parallels Cognition and Behavior in Williams Syndrome

  2004cited by this paper
• Folding of the tectal cortex by local remodeling of neural differentiation

  2004cited by this paper
• The functional neuroanatomy of the human orbitofrontal cortex: evidence from neuroimaging and neuropsychology.

  2004cited by this paper
• LIMK1 and CLIP-115: linking cytoskeletal defects to Williams syndrome.

  2004cited by this paper
• Towards a quantitative, probabilistic neuroanatomy of cerebral cortex.

  2004cited by this paper
• Using case study comparisons to explore genotype-phenotype correlations in Williams-Beuren syndrome

  2003cited by this paper
• Partial deletion of the critical 1.5 Mb interval in Williams-Beuren syndrome

  2003cited by this paper
• Neural Correlates of Auditory Perception in Williams Syndrome: An fMRI Study

  2003cited by this paper
• Lesions of the Orbitofrontal but not Medial Prefrontal Cortex Disrupt Conditioned Reinforcement in Primates

  2003cited by this paper
• Williams Syndrome: 15 Years of Psychological Research

  2003cited by this paper
• The Emotional Brain, Fear, and the Amygdala

  2003cited by this paper
• Interindividual differences in functional interactions among prefrontal, parietal and parahippocampal regions during working memory.

  2003cited by this paper
• GTF2I hemizygosity implicated in mental retardation in Williams syndrome: Genotype–phenotype analysis of five families with deletions in the Williams syndrome region

  2003cited by this paper
• The neural basis of object perception.

  2003cited by this paper
• The BDNF val66met polymorphism affects activity-dependent secretion of BDNF and human memory and hippocampal function.

  2003cited by this paper
• Visuospatial Cognition in Williams Syndrome: Reviewing and Accounting for the Strengths and Weaknesses in Performance

  2003cited by this paper
• Cognitive neuroscience: Cognitive neuroscience of human social behaviour

  2003cited by this paper
• Williams syndrome deficits in visual spatial processing linked to GTF2IRD1 and GTF2I on Chromosome 7q11.23

  2003cited by this paper
• The DNA sequence of human chromosome 7

  2003cited by this paper
• Anxiety, Fears, and Phobias in Persons With Williams Syndrome

  2003cited by this paper
• Neurobiological Models of Visuospatial Cognition in Children With Williams Syndrome: Measures of Dorsal-Stream and Frontal Function

  2003cited by this paper
• Mutational mechanisms of Williams-Beuren syndrome deletions.

  2003cited by this paper
• Distinctive Personality Characteristics of 8-, 9-, and 10-Year-Olds With Williams Syndrome

  2003cited by this paper
• The Roles of Verbal Short-Term Memory and Working Memory in the Acquisition of Grammar by Children With Williams Syndrome

  2003cited by this paper
• Dethroning the Myth: Cognitive Dissociations and Innate Modularity in Williams Syndrome

  2003cited by this paper
• Morphological Abilities of Hebrew-Speaking Adolescents With Williams Syndrome

  2003cited by this paper
• Brain activation to phobia-related pictures in spider phobic humans: an event-related functional magnetic resonance imaging study.

  2003cited by this paper
• Facial expression recognition in Williams syndrome.

  2003cited by this paper
• ERP abnormalities of illusory contour perception in Williams Syndrome

  2003cited by this paper
• Language Abilities of Individuals with Williams Syndrome

  2003cited by this paper
• Generation and Comparative Analysis of ∼3.3 Mb of Mouse Genomic Sequence Orthologous to the Region of Human Chromosome 7q11.23 Implicated in Williams Syndrome

  2002cited by this paper
• Generation and comparative analysis of approximately 3.3 Mb of mouse genomic sequence orthologous to the region of human chromosome 7q11.23 implicated in Williams syndrome.

  2002cited by this paper
• Abnormal spine morphology and enhanced LTP in LIMK-1 knockout mice.

  2002cited by this paper
• The Amygdala Response to Emotional Stimuli: A Comparison of Faces and Scenes

  2002cited by this paper
• Genomic organization of the genes Gtf2ird1, Gtf2i, and Ncf1 at the mouse chromosome 5 region syntenic to the human chromosome 7q11.23 Williams syndrome critical region.

  2002cited by this paper
• Neuronal Size and Neuronal-Packing Density in Primary Visual Cortex

  2002cited by this paper
• The primate amygdala and the neurobiology of social behavior: implications for understanding social anxiety.

  2002cited by this paper
• A study of relative clauses in Williams syndrome

  2002cited by this paper
• Williams syndrome: neuronal size and neuronal-packing density in primary visual cortex.

  2002cited by this paper
• Neuronal and glial metabolite content of the epileptogenic human hippocampus

  2002cited by this paper
• Morphology and morphometry of the corpus callosum in Williams syndrome: A T1-weighted MRI study

  2002cited by this paper
• Increased gyrification in Williams syndrome: evidence using 3D MRI methods.

  2002cited by this paper
• Lexical skills in Williams Syndrome: a cognitive neuropsychological analysis

  2002cited by this paper
• Cerebellar abnormalities in infants and toddlers with Williams syndrome

  2002cited by this paper
• Targeted mutation of Cyln2 in the Williams syndrome critical region links CLIP-115 haploinsufficiency to neurodevelopmental abnormalities in mice

  2002cited by this paper
• Prevalence Estimation of Williams Syndrome

  2002cited by this paper
• Face and place processing in Williams syndrome: evidence for a dorsal-ventral dissociation

  2002cited by this paper
• Corpus callosum morphology of Williams syndrome: relation to genetics and behavior.

  2001cited by this paper
• Speaking of learning How do we acquire our marvellous facility for expressing ourselves in words ?

  2001cited by this paper
• Integration of a c-myc transgene results in disruption of the mouse Gtf2ird1 gene, the homologue of the human GTF2IRD1 gene hemizygously deleted in Williams-Beuren syndrome.

  2001cited by this paper
• Face processing occurs outside the fusiform 'face area' in autism: evidence from functional MRI.

  2001cited by this paper
• Dorsal forebrain anomaly in Williams syndrome.

  2001cited by this paper
• Visual and visuospatial development in young children with Williams syndrome

  2001cited by this paper
• Music Skills and the Expressive Interpretation of Music in Children with Williams-Beuren Syndrome: Pitch, Rhythm, Melodic Imagery, Phrasing, and Musical Affect

  2001cited by this paper
• A 1.5 million–base pair inversion polymorphism in families with Williams-Beuren syndrome

  2001cited by this paper
• Williams syndrome and deficiency in visuospatial recognition.

  2001cited by this paper
• The neurobiology of social cognition.

  2001cited by this paper
• WBSCR14, a gene mapping to the Williams--Beuren syndrome deleted region, is a new member of the Mlx transcription factor network.

  2001cited by this paper
• Speaking of learning

  2001cited by this paper

• Gtf2i-encoded transcription factor Tfii-i regulates myelination via Sox10 and Mbp regulatory elements

  2025cites this paper
• Anxiety in Young Children with Williams Syndrome: A Longitudinal Study

  2025cites this paper
• A Del(5Ncf1-Fkbp6) mouse model of Williams syndrome shows coronary, aortic, and cerebral vascular abnormalities with behavioral deficits

  2025cites this paper
• A long-deletion mouse model of Williams syndrome reveals Ncf1-dependent modulation of vascular and neural phenotypes

  2025influential citation
• Characteristics of Early Language Development in Children With Williams Syndrome

  2025cites this paper
• Neuronal Actin Remodeling and Its Role in Higher Nervous Activity

  2025cites this paper
• [Characteristics of early cognitive development in children with Williams syndrome: a prospective cohort study].

  2024cites this paper
• Syntaxin1A overexpression and pain insensitivity in individuals with 7q11.23 duplication syndrome

  2024cites this paper
• The role of social motivation in sharing and fairness: insights from Williams syndrome

  2024cites this paper
• Examining of Social-Emotional and Moral Characteristics of a Child with Williams Syndrome and a Typically Developing Peer

  2024cites this paper
• Contrasting neurofunctional correlates of face- and visuospatial-processing in children and adolescents with Williams syndrome: convergent results from four fMRI paradigms

  2024cites this paper
• RNA 5-Methylcytosine Modification: Regulatory Molecules, Biological Functions, and Human Diseases

  2024cites this paper
• Social feedback enhances learning in Williams syndrome

  2023cites this paper
• Williams-Beuren's Syndrome: A Case Report in Prince Sultan Military City, Riyadh, Saudi Arabia 2022

  2023cites this paper
• Dissociable Cognitive Systems for Recognizing Places and Navigating through Them: Developmental and Neuropsychological Evidence

  2023influential citation
• Transposons in the Williams–Beuren Syndrome Critical Region are Associated with Social Behavior in Assistance Dogs

  2023cites this paper
• Dorsal visual stream and LIMK1: hemideletion, haplotype, and enduring effects in children with Williams syndrome

  2023cites this paper
• Powering the social brain: Mitochondria in social behaviour.

  2023cites this paper
• Profiling Genome-Wide DNA Methylation in Children with Autism Spectrum Disorder and in Children with Fragile X Syndrome

  2022cites this paper
• Innate frequency-discrimination hyperacuity in Williams-Beuren syndrome mice.

  2022cites this paper
• Clinical phenotypes study of 231 children with Williams syndrome in China: A single‐center retrospective study

  2022cites this paper
• Gene mapping methodology powered by induced genome rearrangements

  2022cites this paper
• Altered White Matter and microRNA Expression in a Murine Model Related to Williams Syndrome Suggests That miR-34b/c Affects Brain Development via Ptpru and Dcx Modulation

  2022cites this paper
• Effects of spatial boundaries on episodic memory development.

  2022cites this paper
• Atypical deletion of Williams–Beuren syndrome reveals the mechanism of neurodevelopmental disorders

  2022cites this paper
• In individuals with Williams syndrome, dysregulation of methylation in non-coding regions of neuronal and oligodendrocyte DNA is associated with pathology and cortical development

  2022cites this paper
• Neuroanatomical correlates of social approach in Williams Syndrome and down syndrome.

  2022cites this paper
• An EEG investigation of alpha and beta activity during resting states in adults with Williams syndrome

  2021cites this paper
• Evaluation and Treatment of Pediatric Calcium Disorders

  2021cites this paper
• Oxytocin receptor activation does not mediate associative fear deficits in a Williams Syndrome model

  2021cites this paper
• Oxytocin and Oxytocin Receptor Gene Regulation in Williams Syndrome: A Systematic Review

  2021cites this paper
• Stereotypies

  2021cites this paper
• Focus on autism and other neurodevelopmental disorders

  2021cites this paper
• Life and Death of Immature Neurons in the Juvenile and Adult Primate Amygdala

  2021cites this paper
• Are feralization and domestication truly mirror processes?

  2021cites this paper
• Sleep's role in memory consolidation: What can we learn from atypical development?

  2021cites this paper
• A Critical Review of Spatial Abilities in Down and Williams Syndromes: Not All Space Is Created Equal

  2021cites this paper
• Williams syndrome

  2021cites this paper
• White matter alterations in Williams syndrome related to behavioral and motor impairments

  2020cites this paper
• An Exploration of Social Cognition in Children with Different Degrees of Genetic Deletion in Williams Syndrome

  2020cites this paper
• Characterizing vulnerable brain areas and circuits in mouse models of autism: Towards understanding pathogenesis and new therapeutic approaches.

  2020cites this paper
• Psychopathology from an Evolutionary Perspective

  2020cites this paper
• The development of visuospatial processing

  2020cites this paper
• Gait characteristics of children with Williams syndrome with impaired visuospatial recognition: a three-dimensional gait analysis study

  2020cites this paper
• Social, neurodevelopmental, endocrine, and head size differences associated with atypical deletions in Williams–Beuren syndrome

  2020cites this paper
• Functions of Gtf2i and Gtf2ird1 in the developing brain: transcription, DNA-binding, and long term behavioral consequences.

  2020cites this paper
• Atypical Deletion of Williams-beuren Syndrome Reveals the Mechanism of Neurodevelopmental Disorders

  2020cites this paper
• Anesthetic Considerations for Patients With Williams Syndrome.

  2020cites this paper
• Dental-craniofacial manifestation and treatment of rare diseases

  2019cites this paper
• Williams syndrome hemideletion and LIMK1 variation both affect dorsal stream functional connectivity.

  2019cites this paper
• Williams syndrome

  2019cites this paper
• An Appraisal of the Influence of the Metabotropic Glutamate 5 (mGlu5) Receptor on Sociability and Anxiety

  2019cites this paper
• La navigation spatiale chez les personnes avec un syndrome de Williams

  2019cites this paper
• Impaired behavioral and neural representation of scenes in Williams syndrome.

  2019cites this paper
• THE FRAGILE BIOLOGY OF SOCIAL GENES AND THE EVOLUTION OF HUMAN SOCIETIES

  2019cites this paper
• Prenatal diagnosis of chromosomal aberrations by chromosomal microarray analysis in fetuses with ultrasound anomalies in the urinary system

  2019cites this paper
• Altered Neocortical Dynamics in a Mouse Model of Williams–Beuren Syndrome

  2019cites this paper
• Gtf2i and Gtf2ird1 mutation are not sufficient to reproduce mouse phenotypes caused by the Williams Syndrome critical region

  2019cites this paper
• Gtf2i and Gtf2ird1 mutation do not account for the full phenotypic effect of the Williams syndrome critical region in mouse models.

  2019influential citation
• Metabotropic Glutamate Receptors and Neurological/Psychiatric Disorders

  2019cites this paper
• Determining the Genetic Contributions of the Williams Syndrome Critical Region to Behavior Using Mouse Models and Human Genetics

  2019cites this paper
• Exhaustivity: Fundamentally Linguistic?

  2019cites this paper
• for recognizing places and navigating through them: causal and developmental evidence

  2019cites this paper
• Functions of Gtf2i and Gtf2ird1 in the developing brain: transcription, DNA-binding, and long term behavioral consequences

  2019cites this paper
• Genes dysregulated in the blood of people with Williams syndrome are enriched in protein-coding genes positively selected in humans.

  2019cites this paper
• Expression of the RNA methyltransferase Nsun5 is essential for developing cerebral cortex

  2019cites this paper
• Attentional rather than sensory differences characterize auditory processing in Williams syndrome

  2018cites this paper
• The 7q11.23 Protein DNAJC30 Interacts with ATP Synthase and Links Mitochondria to Brain Development.

  2018cites this paper
• Activity of Genes with Functions in Human Williams–Beuren Syndrome Is Impacted by Mobile Element Insertions in the Gray Wolf Genome

  2018cites this paper
• The Ontogeny of Human Self-Consciousness

  2018cites this paper
• Adaptive Functioning in Williams Syndrome: A Systematic Review

  2018cites this paper
• The Development of the PRISM Measurement Scales THE PRISM MODEL AND THE DEVELOPMENT OF ITS MEASUREMENT SCALES

  2018cites this paper
• Characteristics of auditory evaluation in Williams syndrome: a systematic review.

  2018cites this paper
• Dissociable spatial memory systems revealed by typical and atypical human development.

  2018cites this paper
• Low-Resolution Place and Response Learning Capacities in Down Syndrome

  2018cites this paper
• A method for determining haploid and triploid genotypes and their association with vascular phenotypes in Williams syndrome and 7q11.23 duplication syndrome

  2018cites this paper
• Cognitive, Behavioral, and Adaptive Profiles in Williams Syndrome With and Without Loss of GTF2IRD2

  2018cites this paper
• Impact of Spaceflight and Artificial Gravity on the Mouse Retina: Biochemical and Proteomic Analysis

  2018cites this paper
• Epigallocatechin-3-gallate improves cardiac hypertrophy and short-term memory deficits in a Williams-Beuren syndrome mouse model

  2018cites this paper
• Quantifier comprehension is linked to linguistic rather than to numerical skills. Evidence from children with Down syndrome and Williams syndrome

  2018cites this paper
• Quantifying the Effects of 16p11.2 Copy Number Variants on Brain Structure: A Multisite Genetic-First Study.

  2018cites this paper
• Autistic traits, resting-state connectivity, and absolute pitch in professional musicians: shared and distinct neural features

  2018cites this paper
• Williams Syndrome

  2018cites this paper
• Williams Syndrome

  2018cites this paper
• Williams Syndrome neuroanatomical score associates with GTF2IRD1 in large-scale magnetic resonance imaging cohorts: a proof of concept for multivariate endophenotypes

  2018cites this paper
• The role of TGF-&bgr; superfamily signaling in neurological disorders

  2018influential citation
• Social Rewards: From Basic Social Building Blocks to Complex Social Behavior

  2018cites this paper
• Linguistic phenotype in a sample of Arabic speaking children with Williams and fragile X syndromes

  2018cites this paper
• Increased glia density in the caudate nucleus in williams syndrome: Implications for frontostriatal dysfunction in autism

  2018cites this paper
• Williams Syndrome, Human Self-Domestication, and Language Evolution

  2018cites this paper
• William’s Syndrome: Brain-Behavior Relationships and Implications for School Psychologist

  2018influential citation
• The phenomenology of temper outbursts in intellectual disabilities

  2017cites this paper
• An event related potential study of inhibitory and attentional control in Williams syndrome adults

  2017cites this paper
• Intellectual Disabilities and Global Developmental Delay

  2017cites this paper
• A Common Polymorphism in a Williams Syndrome Gene Predicts Amygdala Reactivity and Extraversion in Healthy Adults.

  2017cites this paper
• Social Cognition in Individuals With Intellectual and Developmental Disabilities: Recent Advances and Trends in Research

  2017cites this paper
• Increased overall cortical connectivity with syndrome specific local decreases suggested by atypical sleep-EEG synchronization in Williams syndrome

  2017cites this paper
• Common Variation in the GTF2I Gene: A Promising Neurogenetic Mechanism for Affiliative Drive and Social Anxiety.

  2017cites this paper
• Understanding Strategic Information Use During Emotional Expression Judgments in Williams Syndrome

  2017cites this paper
• Williams syndrome deletions and duplications: Genetic windows to understanding anxiety, sociality, autism, and schizophrenia

  2017cites this paper