title: LGI Proteins and Epilepsy in Human and Animals.
abstract: Leucine-rich glioma-inactivated (LGI) protein was first thought to have a suppressor effect in the formation of some cancers. Developments in physiology and medicine made it possible to characterize the function of the LGI protein family and its crucial role in different conditions more precisely. These proteins play an important role in synaptic transmission, and dysfunction may cause hyperexcitability. Genetic mutation of LGI1 was confirmed to be the cause of autosomal dominant lateral temporal lobe epilepsy in humans. The LGI2 mutation was identified in benign familial juvenile epilepsy in Lagotto Romagnolo (LR) dogs. Cats with familial spontaneous temporal lobe epilepsy have been reported, and the etiology might be associated with LGI protein family dysfunction. In addition, an autoimmune reaction against LGI1 was detected in humans and cats with limbic encephalitis. These advances prompted a review of LGI protein function and its role in different seizure disorders.
authors.affiliation: University Clinic of Small Animals, University of Veterinary Medicine, Vienna, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Pakozdy
authors.affiliation: Institute of Immunology, University of Veterinary Medicine, Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Patzl
authors.affiliation: Unit of Physiology and Biophysics, University of Veterinary Medicine, Vienna, Austria.
authors.forename: L
authors.initials: L
authors.lastname: Zimmermann
authors.affiliation: Department of Equine and Small Animal Medicine, University of Helsinki, Helsinki, Finland.
authors.forename: T S
authors.initials: TS
authors.lastname: Jokinen
authors.affiliation: University Clinic of Small Animals, University of Veterinary Medicine, Vienna, Austria.
authors.forename: U
authors.initials: U
authors.lastname: Glantschnigg
authors.affiliation: Epilepsy Center, National Institute of Clinical Neurosciences, Budapest, Hungary.
authors.forename: A
authors.initials: A
authors.lastname: Kelemen
authors.affiliation: Department of Clinical Veterinary Medicine, Nippon Veterinary and Life Science University, Musashinoshi, Tokyo, Japan.
authors.forename: D
authors.initials: D
authors.lastname: Hasegawa
mesh_terms: D000818:Animals; D002371:Cat Diseases / Q000209:etiology / Q000235:genetics; D002415:Cats; D004283:Dog Diseases / Q000209:etiology / Q000235:genetics; D004285:Dogs; D004827:Epilepsy / Q000209:etiology* / Q000235:genetics / Q000662:veterinary; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D020363:Limbic Encephalitis / Q000276:immunology / Q000662:veterinary; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000502:physiology*; D011506:Proteins / Q000235:genetics / Q000276:immunology / Q000502:physiology; D009435:Synaptic Transmission / Q000502:physiology
chemical_list: D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D009419:Nerve Tissue Proteins; D011506:Proteins
keywords: Autoimmune; Epilepsy; Genetic; LGI