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Decreased Dendritic Branching in Frontal, Motor and Limbic Cortex in Rett Syndrome Compared with Trisomy 21
Abstract
The branching of dendrites of pyramidal neurons in premotor frontal, motor and limbic cortex have been identified by us using Golgi technique to be less in Rett Syndrome (RS) brains than in non-Rett control brains. Decreased dendritic branching per se is not pathognomonic of a particular condition and has been reported in numerous disorders associated with mental retardation. This study was designed to test whether the dendritic alterations in Rett Syndrome are the same or different from the alterations present in Down Syndrome (DS), 1 specific form of mental retardation. Sections from Brodmann's areas 6, 4, 20, 43, 28, and 17 of premotor frontal, motor cortex, inferior temporal gyrus, hippocampal formation and the striate cortex from 16 Rett brains, 9 non-Rett brains and 9 Down's brains were prepared for dendrite analysis using the rapid Golgi technique. Drawings of apical and basilar dendrites of pyramidal neurons from 2 cortical layers and Cal were submitted to Sholl analysis. The analyses of Rett brains were compared with the analyses of the Trisomy 21 brains using the repeated measures analysis of covariance, with age as a covariate. The studies demonstrate in our sample that basal dendrites of layer III and V of frontal, layer IV of subiculum, and layer V of motor cortex and apical dendrites of layer III of frontal cortex have a significantly reduced dendritic arborization in RS compared with Trisomy 21. This study suggests that the cortical distribution of the dendritic alterations is specific for Rett Syndrome, and that the premotor frontal, motor and subicular cortex are preferentially involved in the, as yet, undefined process which affects brain growth and function in RS.
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“In Rett syndrome, a severe NDD with specific cognitive and behavioral features, there is a strong PFC hypofunction with structural abnormalities [267–269].”
From Development of prefrontal cortex (2022) · cites this paper as [268]
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“Overall, RTT leads to widespread synaptic and structural alterations, including reduced neuronal size, simplified dendritic arbors, and decreased spine density across multiple brain regions [5,6].”
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Metadata
- Authors
- Armstrong, D. D., Dunn, K., & Antalffy, B.
- Affiliations
- Baylor College of Medicine (US)
- Corresponding author
- Armstrong, Dawna Duncan
- Venue
- Journal of Neuropathology and Experimental Neurology
- Publisher
- Oxford University Press (OUP)
- Published
- 1998-11
- Volume
- 57
- Issue
- 11
- Pages
- 1013-1017
- ISSN
- 0022-3069 (ISSN-L)
- OpenAlex
- W2052422346
- Cited by
- 137
- References
- 0
- Citations by year
- 2026: 3 · 2025: 3 · 2024: 4 · 2022: 2 · 2021: 4 · 2020: 5 · 2019: 3 · 2018: 3 · 2017: 5 · 2016: 6 · 2015: 5 · 2014: 9 · 2013: 5 · 2012: 11
- FWCI
- 2.67
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Neuropathology & Experimental Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (2p01hd024234-09a1)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (5 P30 HD24064-09/10-P14, P30HD024064, 2 PO1 HD24234-09/11)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
- Keywords
- Rett syndrome, Down syndrome, dendritic branching, pyramidal neurons, frontal cortex, motor cortex, limbic cortex, hippocampal subiculum, primary visual cortex, Sholl analysis, brain growth, intellectual disability
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Dendrites, Down Syndrome, Female, Frontal Lobe, Humans, Limbic System, Motor Cortex, Rett Syndrome
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