A human Dravet syndrome model from patient induced pluripotent stem cells
Summary
Our results indicate a functional decline in Dravet neurons, especially in the GABAergic subtype, which supports previous findings in murine disease models, where loss-of-function in GABAergic inhibition appears to be a main driver in epileptogenesis. Our data indicate that patient-derived iPSCs may serve as a new and powerful research platform for genetic disorders, including the epilepsies.
| Authors | Higurashi N, Uchida T, Lossin C, Misumi Y, Okada Y, Akamatsu W, Imaizumi Y, Zhang B, Nabeshima K, Mori MX, Katsurabayashi S, Shirasaka Y, Okano H, Hirose S |
|---|---|
| Journal | Molecular brain |
| Publication Date | 2013 May 2;6:19 |
| PubMed | 23639079 |
| PubMed Central | PMC3655893 |
| DOI | 10.1186/1756-6606-6-19 |