Lissencephaly
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Researchers at University of California release new data on epilepsy
2009 JUN 29 - (NewsRx.com) -- "Mutations in doublecortin (DCX) are associated with intractable epilepsy in humans, due to a severe disorganization of the neocortex and hippocampus known as classical lissencephaly. However, the basis of the epilepsy in lissencephaly remains unclear," scientists in the United States report. "To address potential functional redundancy with murin Dcx, we targeted one of the closest homologues, doublecortin-like kinase 2 (Dclk2). Here, we report that Dcx; Dclk2-null mice display frequent spontaneous seizures that originate in the hippocampus, with most animals dying in the first few months of life. Elevated hippocampal expression of c-fos and loss of somatostatin-positive interneurons were identified, both known to correlate with epilepsy. Dcx and Dclk2 are coexpressed in developing hippocampus, and, in their absence, there is dosage-dependent disrupted hippocampal lamination associated with a cell-autonomous simplification of pyramidal dendritic arborizations leading to reduced inhibitory synaptic tone," wrote G. Kerjan and colleagues, University of California. The researchers concluded: "These data suggest that hippocampal dysmaturation and insufficient receptive field for inhibitory input may underlie the epilepsy in lissencephaly, and suggest potential therapeutic strategies for controlling epilepsy in these patients." Kerjan and colleagues published their study in Proceedings of the National Academy of Sciences of the United States of America (Mice lacking doublecortin and doublecortin-like kinase 2 display altered hippocampal neuronal maturation and spontaneous seizures. Proceedings of the National Academy of Sciences of the United States of America, 2009;106(16):6766-6771). For additional information, contact J.G. Gleeson, University of California, Neurogenetics Laboratory, Howard Hughes Med Institute, Dept. of Neuroscience, La Jolla, CA 92093, USA. The publisher's contact information for the journal Proceedings of the National Academy of Sciences of the United States of America is: National Acad Sciences, 2101 Constitution Avenue NW, Washington, DC 20418, USA. Keywords: United States, La Jolla, Central Nervous System Disease, Epilepsy, Lissencephaly, Seizures, University of California. This article was prepared by Pain & Central Nervous System Week editors from staff and other reports. Copyright 2009, Pain & Central Nervous System Week via NewsRx.com.
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