Journal article
Anomalous inhibitory circuits in cortical tubers of human tuberous sclerosis complex associated with refractory epilepsy: Aberrant expression of parvalbumin and calbindin-D-28k in dysplastic cortex
Journal of child neurology, v 21(12), pp 1058-1063
01 Dec 2006
PMID: 17156698
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Damage or loss of inhibitory. cortical gamma-aminobutyric acid (GABA)ergic interneurons is associated with impaired inhibitory control of neocortical pyramidal cells, leading to hyperexcitability and epileptogenesis. The calcium binding proteins parvalbumin and calbindin-D-28k-are expressed in subpopulations of GABAergic local circuit neurons in the neocortex and can serve as neuronotypic markers. Parvalbumin and calbindin-D-28k facilitate the neuron's ability to sustain firing and provide neuroprotection. The goal of this study was to assess the hitherto unknown status of inhibitory interneurons in cortical tubers of human tuberous sclerosis complex. Surgically excised cortical tubers from three patients with tuberous sclerosis complex were evaluated immunohistochemically with antibodies to parvalbumin and calbindin-D28k, Cortical specimens from young patients with intractable seizures, including microdysgenesis (n = 3), postischemic cortical scarring (n = 1), porencephaly (n = 1), postictal gliosis (n = 3), and low-grade neuronal or glial tumors (n = 5), were also examined for comparison. In cortical tubers, calcium binding protein immunoreactivities (calbindin-D-28k > parvalbumin) were present in medium- or large-size dysplastic neurons, whereas giant or ballooned cells were parvalbumin or calbindin-D-28k negative. In microdysgenesis, a nearly normal number of parvalbumin-positive neurons and a decreased number of calbindin-D-28k-positive neurons were present. In peritumoral but more so in gliotic cortex, a coordinate decrease of parvalbumin and calbindin-D28k immunoreactivities was present. Our findings indicate that the expression of parvalbumin or calbindin-D28k by subpopulations of dysplastic neurons in cortical tubers is aberrant and denotes dysfunctional inhibitory circuits inept for excitoprotection.
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Details
- Title
- Anomalous inhibitory circuits in cortical tubers of human tuberous sclerosis complex associated with refractory epilepsy: Aberrant expression of parvalbumin and calbindin-D-28k in dysplastic cortex
- Creators
- Ignacio Valencia - St. Christopher's Hospital for ChildrenAgustin LegidoKarina YelinDivya KhuranaSanjeev V. Kothare - St. Christopher's Hospital for ChildrenChristos D. Katsetos - Drexel University
- Publication Details
- Journal of child neurology, v 21(12), pp 1058-1063
- Publisher
- Sage
- Number of pages
- 6
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics
- Web of Science ID
- WOS:000243207000010
- Scopus ID
- 2-s2.0-33846397361
- Other Identifier
- 991019168826304721
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InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Clinical Neurology
- Pediatrics