LGI1: a gene involved in epileptogenesis and glioma progression?
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.
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Institute of Human Genetics, School of Medicine, Ludwig Maximilians-University, Goethestrasse 29, 80336 Munich, Germany. wenli.gu@med.uni-muenchen.de
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Piepoli
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D001932:Brain Neoplasms / Q000235:genetics / Q000503:physiopathology*; D004833:Epilepsy, Temporal Lobe / Q000235:genetics / Q000503:physiopathology*; D005910:Glioma / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D011506:Proteins / Q000235:genetics*
D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins