HARBI1 is a 349 amino acid nuclear and cytoplasmic protein belonging to the HARBI1 family. Members of the HARBI1 family of proteins are highly conserved in humans to various bony fish. Considered a transposase-derived protein, HARBI1 may possess nuclease activity and is expressed in brain, eye, nerve tissue, kidney and lung. HARBI1 utilizes divalent metal cations as cofactors, interacts with NAIF1 and promotes translocation to the nucleus. HARBI1 is encoded by a gene located on human chromosome 11, which houses over 1,400 genes and comprises nearly 4% of the human genome. Jervell and Lange-Nielsen syndrome, Jacobsen syndrome, Niemann-Pick disease, hereditary angioedema and Smith-Lemli-Opitz syndrome are associated with defects in genes that maps to chromosome 11.