ELMOD1 is an ELMO domain containing protein that activates Arl2 GTPase []and possibly other GTPases of the Arf family []. Mutations in the mouse ELMOD1 gene cause cochlear hair cell dysfunction and hearing loss []. There are six ELMO domain containing proteins identified in humans. They can be classified into two subgroups, ELMOs and ELMODs, according to protein size and domain architecture []. ELMOs are approximately twice as large as ELMODs and contain multiple domains. The function of the ELMO domain is not clear. ELMOs and ELMODs seem to have distinct cellular and biochemical functions. ELMOs interact with DOCKs and enhance the GEF activity of DOCKs, while ELMODs may play a general role in integrating signalling pathways controlled by Arls and other GTPases [, ].
