PLAG1 is a proto-oncogene. As PLAG1 is a transcription factor, able to activate transcription through the binding to the consensus sequence GRGGC(N)(6-8)GGG, its ectopic expression presumably results in the deregulation of target genes, leading to uncontrolled cell proliferation. PLAG1 has been shown be a transcriptional activator of IGF2 in several tumor types.

Pathology

 PLAG1 amplification and overexpression in tumors

• pleomorphic adenoma of the salivary glands
• lipoblastoma
• hepatoblastoma (#14695992#)

 PLAG1 rearrangement by translocation

• 8q12 rearrangements in lipoblastoma
• promoter swapping between the genes for zinc finger protein PLAG1 and beta-catenin CTNNB1 in pleiomorphic adenoma with t(3;8)(p21;q12) translocations (#9020842#)

 IGF2 regulator role of PLAG1 in hepatoblastoma (#14695992#)

• PLAG1 has been shown be a transcriptional activator of IGF2 in other tumor types (#14695992#)

References

 Zatkova A, Rouillard JM, Hartmann W, Lamb BJ, Kuick R, Eckart M, von Schweinitz D, Koch A, Fonatsch C, Pietsch T, Hanash SM, Wimmer K. Amplification and overexpression of the IGF2 regulator PLAG1 in hepatoblastoma. Genes Chromosomes Cancer. 2004 Feb;39(2):126-37. PMID: #14695992#