The human FREM3 gene codes for an extracellular matrix protein belonging to the same family as FRAS1, FREM2, and QBRICK/FREM1, which have been implicated in Fraser syndrome—a disorder characterized by severe congenital malformations in the development of the nose, ears, and throat, as well as mental retardation (van Haelst et al., 2007). This evidence concerns the gene FREM2 and Fraser syndrome.