LGI Proteins and Epilepsy in Human and Animals

J Vet Intern Med. 2015 Jul-Aug;29(4):997-1005. doi: 10.1111/jvim.12610. Epub 2015 Jun 1.

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.

Keywords: Autoimmune; Epilepsy; Genetic; LGI.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cat Diseases / etiology
  • Cat Diseases / genetics
  • Cats
  • Dog Diseases / etiology
  • Dog Diseases / genetics
  • Dogs
  • Epilepsy / etiology*
  • Epilepsy / genetics
  • Epilepsy / veterinary
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Limbic Encephalitis / immunology
  • Limbic Encephalitis / veterinary
  • Mutation
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / physiology*
  • Proteins / genetics
  • Proteins / immunology
  • Proteins / physiology
  • Synaptic Transmission / physiology

Substances

  • Intracellular Signaling Peptides and Proteins
  • LGI1 protein, human
  • Nerve Tissue Proteins
  • Proteins