Effects of anti-LGI1 human monoclonal antibodies on mouse behavior, ultrastructure, and ion channel distribution at synapses and axon initial segments
Leucine-rich glioma inactivated 1 (LGI1) is a soluble secreted protein which links Adam22/23 proteins across the synaptic cleft and is also abundant in the axon initial segments of in the hippocampus. Anti-LGI1 autoantibodies have been found in CSF and serum of acquired epilepsy patients with symptoms of limbic encephalitis with amnesia and seizures. Previous studies showed human LGI1 autoantibodies co-precipitated with voltage-gated (VG) potassium channel complexes in rodent tissue. As VG-ion channels help maintain homeostasis in hippocampal action potentials, dysregulation of these channels