Article
Altered flurothyl seizure induction latency, phenotype, and subsequent mortality in a mouse model of juvenile neuronal ceroid lipofuscinosis/batten disease.
Epilepsia - 1 Oct 2002
Kriscenski-Perry Elizabeth, Applegate Craig D, Serour Andrew, Mhyre Timothy R, Leonardo Christopher C, Pearce David A
Abstract excerpt
PURPOSE: Juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease, is a pediatric neurodegenerative disease characterized by vision loss, seizure activity, cognitive decline, and premature death. Discovery of the Batten disease-related gene, CLN3, led to creation of a Cln3 protein-deficient mouse model (Cln3-/-), which recapitulates some of the histopathologic characteristics of the human condition. We...
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