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

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...

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Bibliographic Details
Published in:Cell and tissue research 2018, Vol.371 (1), p.47-54
Main Authors: Thodeson, Drew M., Brulet, Rebecca, Hsieh, Jenny
Format: Article
Language:English
Subjects:
Analysis
Animal models
Animals
Biomedical and Life Sciences
Biomedicine
Cell Culture Techniques
Central nervous system
Disease Models, Animal
Epilepsy
Epilepsy, Temporal Lobe - drug therapy
Epilepsy, Temporal Lobe - genetics
Epilepsy, Temporal Lobe - pathology
Epilepsy, Temporal Lobe - physiopathology
Gene Editing
Genetic engineering
Human Genetics
Humans
Ionizing radiation
Medical treatment
Mice
Mice, Transgenic
Molecular Medicine
Mutation
Neural stem cells
Neural Stem Cells - physiology
Neurogenesis
Neurons
Neurons - metabolism
Organoids - metabolism
Pluripotency
Precision medicine
Proteomics
Review
Rodents
Seizures
Seizures (Medicine)
Stem cell transplantation
Stem cells
Temporal lobe
Transgenic mice
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Summary: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.
ISSN:0302-766X
1432-0878
DOI:10.1007/s00441-017-2675-z