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Publication : Epilepsy-related ligand/receptor complex LGI1 and ADAM22 regulate synaptic transmission.

First Author  Fukata Y Year  2006
Journal  Science Volume  313
Issue  5794 Pages  1792-5
PubMed ID  16990550 Mgi Jnum  J:112818
Mgi Id  MGI:3663801 Doi  10.1126/science.1129947
Citation  Fukata Y, et al. (2006) Epilepsy-related ligand/receptor complex LGI1 and ADAM22 regulate synaptic transmission. Science 313(5794):1792-5
abstractText  Abnormally synchronized synaptic transmission in the brain causes epilepsy. Most inherited forms of epilepsy result from mutations in ion channels. However, one form of epilepsy, autosomal dominant partial epilepsy with auditory features (ADPEAF), is characterized by mutations in a secreted neuronal protein, LGI1. We show that ADAM22, a transmembrane protein that when mutated itself causes seizure, serves as a receptor for LGI1. LGI1 enhances AMPA receptor-mediated synaptic transmission in hippocampal slices. The mutated form of LGI1 fails to bind to ADAM22. ADAM22 is anchored to the postsynaptic density by cytoskeletal scaffolds containing stargazin. These studies in rat brain indicate possible avenues for understanding human epilepsy.
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