NaV1.1 and NaV1.6 selective compounds reduce the behavior phenotype in a novel zebrafish model for Dravet Syndrome

By |2019-07-15T23:23:04-05:00July 14th, 2019|Categories: DSF Research Review|Tags: , , |

Dravet syndrome is caused by loss-of-function mutations in the SCN1A gene, which codes for the alpha subunit of the NaV1.1 sodium channel. However, there are other sodium channels, and it is known that gain-of-function mutations in SCN8A, the gene that codes for the alpha subunit of the NaV1.6 sodium channel, can lead to severe [...]

The Efficacy of Ketogenic Diet in 60 Chinese Patients With Dravet Syndrome

By |2019-07-15T23:17:09-05:00July 13th, 2019|Categories: DSF Research Review|Tags: , , |

This was a retrospective study, gathering information on Dravet patients who, over the last nine years, had followed a strict ketogenic diet for at least 12 weeks. In total, there were 60 children identified. The duration of their ketogenic diets ranged from 12 weeks to 54 months, with a median duration of 44 weeks. [...]

Potentiating α2 subunit containing perisomatic GABAA receptors protects against seizures in a mouse model of Dravet syndrome

By |2019-06-10T12:53:23-05:00June 7th, 2019|Categories: DSF Research Review|Tags: , , |

Many of the drugs used to treat Dravet Syndrome work by interacting with GABAA receptors.  Each GABAA receptor is composed of subunits, and there are different versions of receptors depending on which subunits they have. Drugs have unwanted side-effects, and it is worth asking: if we hit just one specific kind of GABAA receptor, [...]

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