ELMOD2 is a 293 amino acid protein that is one of six human proteins that contain an ELMO domain. ELMOD2 is thought to interact with DOCK1 through a SH3-binding site, which enhances the guanine nucleotide exchange factor (GEF) activity of DOCK1. Therefore, ELMOD2 may also be part of a complex with DOCK1 and Rac1 that is required to activate Rac Rho small GTPases. Evidence of decreased ELMOD2 expression in lung of individuals with familial idiopathic pulmonary fibrosis (IPF) suggests that defects in the gene encoding ELMOD2 may be linked to susceptibility for familial IPF.