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Decreased density of cholinergic interneurons in striatal territories in Williams syndrome.
Hanson, Kari L; Lew, Caroline H; Hrvoj-Mihic, Branka; Cuevas, Deion; Greiner, Demi M Z; Groeniger, Kimberly M; Edler, Melissa K; Halgren, Eric; Bellugi, Ursula; Raghanti, Mary Ann; Semendeferi, Katerina.
Affiliation
  • Hanson KL; Department of Anthropology, University of California San Diego, La Jolla, USA.
  • Lew CH; Institute for Neural Computation, University of California San Diego, La Jolla, USA.
  • Hrvoj-Mihic B; Department of Anthropology, University of California San Diego, La Jolla, USA.
  • Cuevas D; Department of Anthropology, University of California San Diego, La Jolla, USA.
  • Greiner DMZ; Department of Anthropology, University of California San Diego, La Jolla, USA.
  • Groeniger KM; Department of Anthropology, University of California San Diego, La Jolla, USA.
  • Edler MK; Department of Anthropology, University of California San Diego, La Jolla, USA.
  • Halgren E; Department of Anthropology, School of Biomedical Sciences and Brain Health Research Institute, Kent State University, Kent, USA.
  • Bellugi U; Department of Radiology, University of California San Diego, La Jolla, USA.
  • Raghanti MA; Center for Human Brain Activity Mapping, University of California San Diego, La Jolla, USA.
  • Semendeferi K; Neurosciences Graduate Program, University of California San Diego, La Jolla, USA.
Brain Struct Funct ; 225(3): 1019-1032, 2020 Apr.
Article in En | MEDLINE | ID: mdl-32189114
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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Williams Syndrome / Corpus Striatum / Cholinergic Neurons / Interneurons Limits: Adolescent / Adult / Aged / Female / Humans / Male / Middle aged Language: En Journal: Brain Struct Funct Journal subject: CEREBRO Year: 2020 Document type: Article Affiliation country:

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Williams Syndrome / Corpus Striatum / Cholinergic Neurons / Interneurons Limits: Adolescent / Adult / Aged / Female / Humans / Male / Middle aged Language: En Journal: Brain Struct Funct Journal subject: CEREBRO Year: 2020 Document type: Article Affiliation country:
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