PRAME

Description

The PRAME (PRAME nuclear receptor transcriptional regulator) is a protein-coding gene located on chromosome 22.

PRAME (preferentially expressed antigen of melanoma) is a protein encoded by the PRAME gene in humans. It is primarily expressed in human melanomas and is recognized by cytolytic T lymphocytes. PRAME is not expressed in normal tissues, except for the testis. Its expression pattern is similar to other cancer-testis (CT) antigens, such as MAGE, BAGE, and GAGE, but unlike these other CT antigens, PRAME is also expressed in acute leukemias. The overexpression of PRAME in tumor tissues and its relative low levels in normal somatic tissues make it a potential target for cancer therapy. Immunotherapy approaches involving vaccines and cellular immunotherapies are being investigated for PRAME-specific cancer treatment. PRAME can inhibit retinoic acid signaling and retinoic acid mediated differentiation and apoptosis. PRAME overexpression in triple negative breast cancer has also been found to promote cancer cell motility through induction of the epithelial-to-mesenchymal transition.

PRAME is a substrate-recognition component of a Cul2-RING (CRL2) E3 ubiquitin-protein ligase complex. This complex mediates the ubiquitination and degradation of target proteins, including truncated MSRB1/SEPX1 selenoproteins produced by failed UGA/Sec decoding. In the nucleus, the CRL2(PRAME) complex is recruited to epigenetically and transcriptionally active promoter regions bound by nuclear transcription factor Y (NFY) and may play a role in chromatin regulation. PRAME functions as a transcriptional repressor, inhibiting the signaling of retinoic acid through the retinoic acid receptors RARA, RARB and RARG. This prevents retinoic acid-induced cell proliferation arrest, differentiation, and apoptosis.

PRAME is also known as CT130, MAPE, OIP-4, OIP4.

Associated Diseases

WES Whole Exome Sequencing