The leucine-rich, glioma inactivated gene 2 (LGI2) is a member of the LGI family in which LGI1 is the exemplar. The LGI family consists of four of highly related proteins containing leucine-rich repeats (LRRs) which are highly similar to other transmembrane signaling molecules and receptors. LGI1 has been identified as a candidate tumor suppressor gene for glioma and plays a role in autodominant lateral temporal epilepsy (ADTLE), an epileptic syndrome characterized by focal seizures with predominant auditory symptoms. Despite its high homology with LGI1 and similar pattern of expression, mutations in LGI2 have not been found to be associated with ADTLE.