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Neural stem cells and epilepsy: functional roles and disease-in-a-dish models

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Abstract

Epilepsy is a disorder of the central nervous system characterized by spontaneous recurrent seizures. Although current therapies exist to control the number and severity of clinical seizures, there are no pharmacological cures or disease-modifying treatments available. Use of transgenic mouse models has allowed an understanding of neural stem cells in their relation to epileptogenesis in mesial temporal lobe epilepsy. Further, with the significant discovery of factors necessary to reprogram adult somatic cell types into pluripotent stem cells, it has become possible to study monogenic epilepsy-in-a-dish using patient-derived neurons. This discovery along with some of the newest technological advances in recapitulating brain development in a dish has brought us closer than ever to a platform in which to study and understand the mechanisms of this disease. These technologies will be critical in understanding the mechanism of epileptogenesis and ultimately lead to improved therapies and precision medicine for patients with epilepsy.

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Acknowledgements

We thank Jose Cabrera for help with the figures. This work was supported by grants from the National Institute of Health (NIH) R01NS081203, R01NS089770, R01NS093992 and K02AG041815 to J.H., Department of Defense W81XWH-15-1 to J.H., American Heart Association 15GRNT25750034 to J.H., a grant from the Texas Institute for Brain Injury and Repair to J.H. and an award from the National Center for Advancing Translational Sciences (NIH) UL1TR001105 to D.T.

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  1. Departments of Pediatrics and Neurology and Neurotherapeutics, Division of Child Neurology, UT Southwestern Medical Center, Dallas, TX, 75390, USA

    Drew M. Thodeson

  2. Department of Molecular Biology, UT Southwestern Medical Center, Dallas, TX, 75390, USA

    Rebecca Brulet & Jenny Hsieh

  3. Department of Neurology and Neurotherapeutics, UT Southwestern Medical Center, Dallas, TX, 75390, USA

    Rebecca Brulet & Jenny Hsieh

  4. Hamon Center for Regenerative Science and Medicine, UT Southwestern Medical Center, Dallas, TX, 75390, USA

    Rebecca Brulet & Jenny Hsieh

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  1. Drew M. Thodeson
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Correspondence to Jenny Hsieh.

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Thodeson, D.M., Brulet, R. & Hsieh, J. Neural stem cells and epilepsy: functional roles and disease-in-a-dish models. Cell Tissue Res 371, 47–54 (2018). https://doi.org/10.1007/s00441-017-2675-z

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