PLAG1

Description

The PLAG1 (PLAG1 zinc finger) is a protein-coding gene located on chromosome 8.

PLAG1 is a zinc finger protein encoded by the PLAG1 gene in humans. It contains two potential nuclear localization signals and is developmentally regulated. PLAG1 is frequently rearranged in pleomorphic adenomas of the salivary glands and is activated by chromosomal translocations involving 8q12 in a subset of these tumors. PLAG1 interacts with Karyopherin alpha 2.

PLAG1 is a transcription factor that activates target genes like IGFII, leading to uncontrolled cell growth. Overexpression in cultured cells results in increased proliferation and transformation. Other targets like CRLF1, CRABP2, CRIP2, and PIGF are also strongly induced by PLAG1. PLAG1 is a proto-oncogene whose abnormal expression can cause pleomorphic adenomas of the salivary gland and lipoblastomas. Increased PLAG1 is linked to higher IGFII levels and is often seen in hepatoblastoma, a common childhood liver cancer. PLAG1 collaborates with CBFB-MYH11, a fusion gene involved in myeloid leukemia.

PLAG1 is also known as PSA, SGPA, SRS4, ZNF912.

Associated Diseases

• Silver-Russell syndrome 4
• Silver-Russell syndrome due to a point mutation
• Salivary gland adenoma, pleomorphic