Pleiomorphic adenoma gene 1 protein (PLAG1) is a transcription factor whose activation results in up-regulation of target genes, such as IGFII, leading to uncontrolled cell proliferation. When PLAG1 overexpression in cultured cells, higher proliferation rate and transformation are observed. Other target genes such as CRLF1, CRABP2, CRIP2, PIGF are strongly induced in cells with PLAG1 induction. PLAG1 ectopic expression can trigger the development of pleomorphic adenomas of the salivary gland and lipoblastomas. PLAG1 overexpression is associated with up-regulation of IGFII, is frequently observed in hepatoblastoma, common primary liver tumor in childhood. PLAG1 cooperates with CBFB-MYH11, a fusion gene important for myeloid leukemia.
