Basal Dendritic Morphology of Cortical Pyramidal Neurons in Williams Syndrome: Prefrontal Cortex and Beyond
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Williams syndrome (WS) is a unique neurodevelopmental disorder with a specific behavioral and cognitive profile, which includes hyperaffiliative behavior, poor social judgment, and lack of social inhibition. Here we examined the morphology of basal dendrites on pyramidal neurons in the cortex of two rare adult subjects with WS. Specifically, we examined two areas in the prefrontal cortex (PFC)-the frontal pole (Brodmann area 10) and the orbitofrontal cortex (Brodmann area 11)-and three areas in the motor, sensory, and visual cortex (BA 4, BA 3-1-2, BA 18). The findings suggest that the morphology of basal dendrites on the pyramidal neurons is altered in the cortex of WS, with differences that were layer-specific, more prominent in PFC areas, and displayed an overall pattern of dendritic organization that differentiates WS from other disorders. In particular, and unlike what was expected based on typically developing brains, basal dendrites in the two PFC areas did not display longer and more branched dendrites compared to motor, sensory and visual areas. Moreover, dendritic branching, dendritic length, and the number of dendritic spines differed little within PFC and between the central executive region (BA 10) and BA 11 that is part of the orbitofrontal region involved into emotional processing. In contrast, the relationship between the degree of neuronal branching in supra- versus infra-granular layers was spared in WS. Although this study utilized tissue held in formalin for a prolonged period of time and the number of neurons available for analysis was limited, our findings indicate that WS cortex, similar to that in other neurodevelopmental disorders such as Down syndrome, Rett syndrome, Fragile X, and idiopathic autism, has altered morphology of basal dendrites on pyramidal neurons, which appears more prominent in selected areas of the PFC. Results were examined from developmental perspectives and discussed in the context of other neurodevelopmental disorders. We have proposed hypotheses for further investigations of morphological changes on basal dendrites in WS, a syndrome of particular interest given its unique social and cognitive phenotype.
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In Williams syndrome, the structural organization of prefrontal pyramidals, specifically their density and dendritic arboring, is severely affected.
“The structural organization of prefrontal pyramidals, specifically their density and dendritic arboring, is severely affected [298, 299].”
From Development of prefrontal cortex (2022) · cites this paper as [298]
The Discussion (page unavailable) reports altered basal dendritic organization in Williams syndrome, more prominent in selected prefrontal areas. It also reports decreased neuronal body density in layers V/VI of BA 10, citing Lew et al. (2017), but says density did not differ significantly in layers II/III. Thus, the claim overstates the breadth and severity of the findings and treats neuronal body density as specifically pyramidal-neuron density. (LLM, conf=0.87)
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Development of prefrontal cortex
Kolk, S. M., & Rakic, P. (2022) · Neuropsychopharmacology · doi:10.1038/s41386-021-01137-9 · Cited by 378 · see citing works
“The structural organization of prefrontal pyramidals, specifically their density and dendritic arboring, is severely affected [298, 299].”
Background
- Authors
- Hrvoj-Mihic, B., Hanson, K. L., Lew, C. H., Stefanacci, L., Jacobs, B., Bellugi, U., & Semendeferi, K.
- Affiliations
- University of California San Diego (US)
- Colorado College (US)
- Salk Institute for Biological Studies (US)
- Corresponding author
- Semendeferi, Katerina
- Venue
- Frontiers in Neuroscience
- Publisher
- Frontiers Media SA
- Published
- 2017-08-11
- Volume
- 11
- Pages
- 419-419
- ISSN
- 1662-453X (ISSN-L)
- PMC
- PMC5554499
- OpenAlex
- W2744977013
- Cited by
- 21
- References
- 61
- Citations by year
- 2026: 1 · 2024: 1 · 2023: 4 · 2022: 2 · 2021: 2 · 2020: 3 · 2019: 3 · 2018: 3 · 2017: 2
- FWCI
- 1.27
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by
- Locations
- Frontiers in Neuroscience (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Institute of Mental Health (NIMH R56MH109587)
- National Institutes of Health (R03MH103697)
- Topics
- Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Keywords
- Williams syndrome, basal dendrites, pyramidal neurons, dendritic morphology, prefrontal cortex, frontal pole, orbitofrontal cortex, cortical layers, neurodevelopmental disorders, dendritic branching, dendritic spines, social behavior
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