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In Vivo Targeting of Neural Progenitor Cells in Ferret Neocortex by In Utero Electroporation
Published on: May 6, 2020
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Pathophysiological analyses of cortical malformation using gyrencephalic mammals.
Kosuke Masuda1,2,3, Tomohisa Toda1,2,3, Yohei Shinmyo1,2
1Department of Biophysical Genetics, Graduate School of Medical Sciences, Kanazawa University, Ishikawa 920-8640, Japan.
Scientific Reports
|October 21, 2015
Summary
Researchers modeled thanatophoric dysplasia (TD) in ferrets, revealing that fibroblast growth factor 8 causes megalencephaly and polymicrogyria by increasing outer radial glial cells and progenitor cells.
Area of Science:
- Neuroscience
- Developmental Biology
- Genetics
Background:
- Cortical gyri are key features of higher mammal brains.
- Malformations of cortical gyri lead to severe neurological disabilities.
- Understanding the mechanisms of cortical malformations is crucial.
Purpose of the Study:
- To investigate the molecular mechanisms underlying cortical malformations.
- To create a ferret model for studying thanatophoric dysplasia (TD).
- To explore the role of fibroblast growth factor 8 (FGF8) in cortical development.
Main Methods:
- Utilized gyrencephalic carnivore ferrets.
- Employed genetic manipulation via in utero electroporation.
- Expressed fibroblast growth factor 8 (FGF8) in the ferret cerebral cortex.
Main Results:
- Successfully recapitulated cortical phenotypes of thanatophoric dysplasia (TD).
- The TD ferret model exhibited megalencephaly and polymicrogyria.
- Observed a marked increase in outer radial glial cells (oRGs) and intermediate progenitor cells (IPs).
Conclusions:
- Increased oRGs and IPs may underlie the pathogenesis of polymicrogyria.
- FGF8 signaling is implicated in cortical malformations.
- Findings provide insights into the molecular mechanisms of cortical gyrus formation and malformation.
