DSF Research Review
Welcome to DSF’s Research Review! We use this space to keep you up to date on the most exciting studies that are published each month on Dravet syndrome and SCN1A. Each review includes a link to the original abstract or article (where possible), as well as a lay-person’s summary. Have questions? Email our team.
Looking for more information on research? Click on the button below to go to a list of pubmed published scientific articles on Dravet syndrome.
April 2020
Fenfluramine acts as a positive modulator of sigma-1 receptors
In previous posts we have profiled fenfluramine as a recent promising add-on therapy for Dravet syndrome, which not only reduces seizures but also improves executive functioning (behavior, emotions, and cognition). Fenfluramine's known pharmacological profile [...]
The Glucagon-Like Peptide-1 Analogue Liraglutide Reduces Seizures Susceptibility, Cognition Dysfunction and Neuronal Apoptosis in a Mouse Model of Dravet Syndrome
Glucagon-like peptide-1 (GLP-1) is a hormone that is secreted from intestinal cells after food ingestion, stimulates insulin secretion, and helps regulate blood sugar levels. It also binds to receptors in the brain and promotes [...]
March 2020
New insights into the early mechanisms of epileptogenesis in a zebrafish model of Dravet syndrome
This study used zebrafish to try to gain insights into the very earliest neurodevelopmental effects occurring in Dravet syndrome. In previous posts we have explained that appropriately mutated zebrafish are now an established [...]
February 2020
SCN8A Antisense Oligonucleotide is Protective in Mouse Models of SCN8A Encephalopathy and Dravet Syndrome
Dravet Syndrome is caused by loss-of-function mutations affecting the NaV1.1 sodium channel. In a July 2019 research summary, we pointed out that gain-of-function mutations in another sodium channel, called NaV1.6, can lead to severe [...]
Early EEG and behavioral alterations in Dravet mice
This study explored EEG characteristics over the life cycle of Dravet mice; that is, through the febrile, worsening, and stabilization stages. One common way to analyze EEG spectra is to compute the "power spectral [...]
Dravet syndrome: Treatment options and management of prolonged seizures
The journal Epilepsia has devoted a Special Issue to "Dravet Syndrome and other Sodium Channel Related Encephalopathies". It is Volume 60, Issue S3, and can be accessed online - it contains many good articles, [...]
January 2020
Sexually Divergent Mortality and Partial Phenotypic Rescue after Gene Therapy in a Mouse Model of Dravet Syndrome
Dravet syndrome is caused by a mutation in the SCN1A gene, which codes for the NaV1.1 sodium channel a subunit. As described in previous summaries, gene therapy is becoming a very promising approach for [...]
Profound Reduction in Seizure Frequency (≥75%) Leads to Improved Everyday Executive Function: Analysis From a Phase 3 Study of ZX008 (Fenfluramine HCl) in Children/Young Adults With Dravet Syndrome
This month my literature survey returned a poster that was presented at the recent American Epilepsy Society meeting. Although this is not a peer-reviewed journal article, I include it because it is interesting, and [...]