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Lipoblastoma : PLAG1

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Molecular Pathogenesis: PLAG1 (Pleomorphic Adenoma Gene 1), located on 8q12.1, is a proto-oncogene whose ectopic expression underlies the pathogenesis of lipoblastomas and pleomorphic adenomas of the salivary gland. The gene encodes a 500 amino acid zinc finger protein (74 kDa) that acts as a transcription factor. Overexpression results in up-regulation of target genes, such as IGFII, leading to uncontrolled cell proliferation. Other target genes such as CRLF1, CRABP2, CRIP2, PIGF are strongly induced in cells with PLAG1 induction.

Image source: David Gisselsson, Atlas of Genetics and Cytogenetics in Oncology and Haematology. Image used under: Creative Commons Attribution-NonCommercial No Derivative Works 2.0 France License.

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