Neurogenetics

, Volume 6, Issue 2, pp 59–66

LGI1: a gene involved in epileptogenesis and glioma progression?

  • W. Gu
  • E. Brodtkorb
  • T. Piepoli
  • G. Finocchiaro
  • O. K. Steinlein
Review Article

DOI: 10.1007/s10048-005-0216-5

Cite this article as:
Gu, W., Brodtkorb, E., Piepoli, T. et al. Neurogenetics (2005) 6: 59. doi:10.1007/s10048-005-0216-5

Abstract

The leucine-rich, glioma inactivated gene 1 (LGI1) gene on human chromosome 10q24 was first identified as a candidate tumor suppressor gene for glioma. Surprisingly, mutations in LGI1 were also shown to cause an idiopathic epilepsy syndrome, autosomal dominant lateral temporal lobe epilepsy (ADLTE). LGI1 is one of the only two currently known non-ion channel genes whose mutations cause idiopathic epilepsy in humans. In this review we summarize the current data on structure and function of the LGI1 protein and discuss clinical aspects of ADLTE and their correlation with LGI1. We also propose that the evidence supporting the tumor suppressor role of LGI1 in malignant gliomas is weak and that further work is necessary to establish LGI1 role in glial cells.

Keywords

LGI1Lateral temporal epilepsyGliomaExtracellular matrixLRR protein

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • W. Gu
    • 1
  • E. Brodtkorb
    • 2
  • T. Piepoli
    • 3
  • G. Finocchiaro
    • 3
  • O. K. Steinlein
    • 1
  1. 1.Institute of Human Genetics, School of MedicineLudwig Maximilians-UniversityMunichGermany
  2. 2.Department of NeurologyTrondheim University HospitalTrondheimNorway
  3. 3.Laboratory of Neuro-Oncology and Gene TherapyIstituto Nazionale Neurologico BestaMilanItaly