Article
Genetic background modulates impaired excitability of inhibitory neurons in a mouse model of Dravet syndrome.
Neurobiology of disease - 1 Jan 2015
Rubinstein Moran, Westenbroek Ruth E, Yu Frank H, Jones Christina J, Scheuer Todd, Catterall William A
Abstract excerpt
Dominant loss-of-function mutations in voltage-gated sodium channel NaV1.1 cause Dravet Syndrome, an intractable childhood-onset epilepsy. NaV1.1(+/-) Dravet Syndrome mice in C57BL/6 genetic background exhibit severe seizures, cognitive and social impairments, and premature death. Here we show that Dravet Syndrome mice in pure 129/SvJ genetic background have many fewer seizures and much less premature death than...
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