Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complex
Once believed to be the culprits of epileptogenic activity, the functional properties of balloon/giant cells (BC/GC), commonly found in some malformations of cortical development including focal cortical dysplasia type IIb (FCDIIb) and tuberous sclerosis complex (TSC), are beginning to be unraveled....
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Frontiers Media S.A.
2025-01-01
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| Series: | Frontiers in Cellular Neuroscience |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fncel.2024.1486315/full |
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| author | Joyce Zhang Deneen Argueta Xiaoping Tong Harry V. Vinters Gary W. Mathern Carlos Cepeda |
| author_facet | Joyce Zhang Deneen Argueta Xiaoping Tong Harry V. Vinters Gary W. Mathern Carlos Cepeda |
| author_sort | Joyce Zhang |
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| description | Once believed to be the culprits of epileptogenic activity, the functional properties of balloon/giant cells (BC/GC), commonly found in some malformations of cortical development including focal cortical dysplasia type IIb (FCDIIb) and tuberous sclerosis complex (TSC), are beginning to be unraveled. These abnormal cells emerge during early brain development as a result of a hyperactive mTOR pathway and may express both neuronal and glial markers. A paradigm shift occurred when our group demonstrated that BC/GC in pediatric cases of FCDIIb and TSC are unable to generate action potentials and lack synaptic inputs. Hence, their role in epileptogenesis remained obscure. In this review, we provide a detailed characterization of abnormal non-neuronal cells including BC/GC, intermediate cells, and dysmorphic/reactive astrocytes found in FCDIIb and TSC cases, with special emphasis on electrophysiological and morphological assessments. Regardless of pathology, the electrophysiological properties of abnormal cells appear more glial-like, while others appear more neuronal-like. Their morphology also differs in terms of somatic size, shape, and dendritic elaboration. A common feature of these types of non-neuronal cells is their inability to generate action potentials. Thus, despite their distinct properties and etiologies, they share a common functional feature. We hypothesize that, although the exact role of abnormal non-neuronal cells in FCDIIb and TSC remains mysterious, it can be suggested that cells displaying more glial-like properties function in a similar way as astrocytes do, i.e., to buffer K+ ions and neurotransmitters, while those with more neuronal properties, may represent a metabolic burden due to high energy demands but inability to receive or transmit electric signals. In addition, due to the heterogeneity of these cells, a new classification scheme based on morphological, electrophysiological, and gene/protein expression in FCDIIb and TSC cases seems warranted. |
| format | Article |
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| institution | Kabale University |
| issn | 1662-5102 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Cellular Neuroscience |
| spelling | doaj-art-67e0991429b0403da62bb6d394ff2dc02025-01-06T06:59:18ZengFrontiers Media S.A.Frontiers in Cellular Neuroscience1662-51022025-01-011810.3389/fncel.2024.14863151486315Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complexJoyce Zhang0Deneen Argueta1Xiaoping Tong2Harry V. Vinters3Gary W. Mathern4Carlos Cepeda5IDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California - Los Angeles, Los Angeles, CA, United StatesIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California - Los Angeles, Los Angeles, CA, United StatesDepartment of Anatomy and Physiology, Shanghai Jiao Tong University School of Medicine, Shanghai, ChinaDepartment of Pathology and Laboratory Medicine, University of California - Los Angeles, Los Angeles, CA, United StatesDepartment of Neurosurgery, David Geffen School of Medicine, University of California - Los Angeles, Los Angeles, CA, United StatesIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California - Los Angeles, Los Angeles, CA, United StatesOnce believed to be the culprits of epileptogenic activity, the functional properties of balloon/giant cells (BC/GC), commonly found in some malformations of cortical development including focal cortical dysplasia type IIb (FCDIIb) and tuberous sclerosis complex (TSC), are beginning to be unraveled. These abnormal cells emerge during early brain development as a result of a hyperactive mTOR pathway and may express both neuronal and glial markers. A paradigm shift occurred when our group demonstrated that BC/GC in pediatric cases of FCDIIb and TSC are unable to generate action potentials and lack synaptic inputs. Hence, their role in epileptogenesis remained obscure. In this review, we provide a detailed characterization of abnormal non-neuronal cells including BC/GC, intermediate cells, and dysmorphic/reactive astrocytes found in FCDIIb and TSC cases, with special emphasis on electrophysiological and morphological assessments. Regardless of pathology, the electrophysiological properties of abnormal cells appear more glial-like, while others appear more neuronal-like. Their morphology also differs in terms of somatic size, shape, and dendritic elaboration. A common feature of these types of non-neuronal cells is their inability to generate action potentials. Thus, despite their distinct properties and etiologies, they share a common functional feature. We hypothesize that, although the exact role of abnormal non-neuronal cells in FCDIIb and TSC remains mysterious, it can be suggested that cells displaying more glial-like properties function in a similar way as astrocytes do, i.e., to buffer K+ ions and neurotransmitters, while those with more neuronal properties, may represent a metabolic burden due to high energy demands but inability to receive or transmit electric signals. In addition, due to the heterogeneity of these cells, a new classification scheme based on morphological, electrophysiological, and gene/protein expression in FCDIIb and TSC cases seems warranted.https://www.frontiersin.org/articles/10.3389/fncel.2024.1486315/fullfocal cortical dysplasiatuberous sclerosis complexballoon cellselectrophysiologypediatric epilepsy |
| spellingShingle | Joyce Zhang Deneen Argueta Xiaoping Tong Harry V. Vinters Gary W. Mathern Carlos Cepeda Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complex Frontiers in Cellular Neuroscience focal cortical dysplasia tuberous sclerosis complex balloon cells electrophysiology pediatric epilepsy |
| title | Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complex |
| title_full | Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complex |
| title_fullStr | Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complex |
| title_full_unstemmed | Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complex |
| title_short | Iconography of abnormal non-neuronal cells in pediatric focal cortical dysplasia type IIb and tuberous sclerosis complex |
| title_sort | iconography of abnormal non neuronal cells in pediatric focal cortical dysplasia type iib and tuberous sclerosis complex |
| topic | focal cortical dysplasia tuberous sclerosis complex balloon cells electrophysiology pediatric epilepsy |
| url | https://www.frontiersin.org/articles/10.3389/fncel.2024.1486315/full |
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