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Decreased density of cholinergic interneurons in striatal territories in Williams syndrome
- Source :
- Brain Struct Funct, Brain structure & function, vol 225, iss 3
- Publication Year :
- 2019
-
Abstract
- Williams syndrome (WS) is a rare neurodevelopmental disorder caused by the hemideletion of approximately 25-28 genes at 7q11.23. Its unusual social and cognitive phenotype is most strikingly characterized by the disinhibition of social behavior, in addition to reduced global IQ, with a relative sparing of language ability. Hypersociality and increased social approach behavior in WS may represent a unique inability to inhibit responses to specific social stimuli, which is likely associated with abnormalities of frontostriatal circuitry. The striatum is characterized by a diversity of interneuron subtypes, including inhibitory parvalbumin-positive interneurons (PV+) and excitatory cholinergic interneurons (Ch+). Animal model research has identified an important role for these specialized cells in regulating social approach behavior. Previous research in humans identified a depletion of interneuron subtypes associated with neuropsychiatric disorders. Here, we examined the density of PV+ and Ch+ interneurons in the striatum of 13 WS and neurotypical (NT) subjects. We found a significant reduction in the density of Ch+ interneurons in the medial caudate nucleus and nucleus accumbens, important regions receiving cortical afferents from the orbitofrontal and ventromedial prefrontal cortex, and circuitry involved in language and reward systems. No significant difference in the distribution of PV+ interneurons was found. The pattern of decreased Ch+ interneuron densities in WS differs from patterns of interneuron depletion found in other disorders.
- Subjects :
- Male
Williams Syndrome
Medical Physiology
Caudate nucleus
Striatum
0302 clinical medicine
Neurodevelopmental disorder
Basal ganglia
Pediatric
musculoskeletal, neural, and ocular physiology
General Neuroscience
05 social sciences
Middle Aged
Cholinergic Neurons
Mental Health
medicine.anatomical_structure
Parvalbumins
Cognitive Sciences
Female
Anatomy
medicine.symptom
Adult
Histology
Interneuron
Adolescent
Ventromedial prefrontal cortex
Nucleus accumbens
Biology
Basic Behavioral and Social Science
050105 experimental psychology
Article
Choline O-Acetyltransferase
03 medical and health sciences
Young Adult
Clinical Research
Interneurons
Behavioral and Social Science
medicine
Humans
0501 psychology and cognitive sciences
Aged
Neurology & Neurosurgery
Neurosciences
medicine.disease
Corpus Striatum
Brain Disorders
nervous system
Disinhibition
Neuroscience
030217 neurology & neurosurgery
Developmental Biology
Subjects
Details
- ISSN :
- 18632661
- Volume :
- 225
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Brain structurefunction
- Accession number :
- edsair.doi.dedup.....c0f56939bc2661bf722b56ef01c96f2f