Open AccessGlutamate Receptors: Finally Fingered in Inherited Epilepsy?
Author(s) -
Poolos Nicholas P.
Publication year - 2007
Publication title -
epilepsy currents
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.415
H-Index - 22
eISSN - 1535-7511
pISSN - 1535-7597
DOI - 10.1111/j.1535-7511.2007.00191.x
Subject(s) - ampa receptor , epilepsy , neurotransmission , neuroscience , postsynaptic potential , receptor , glutamate receptor , transmembrane protein , medicine , postsynaptic density , biology
Fukata Y, Adesnik H, Iwanaga T, Bredt DS, Nicoll RA, Fukata M. Science 2006;313(5794):1792–1795. 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.