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CRTC1-MAML2在涎腺黏液表皮样癌中的研究进展

Research Progress of CRTC1-MAML2 in Mucoepidermoid Carcinoma of Salivary Gland
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摘要 黏液表皮样癌(MEC)是以黏液细胞、中间细胞和表皮样细胞构成的上皮性恶性肿瘤,是涎腺中最常见的一种恶性肿瘤。目前MEC的致癌机制尚不明确,诊断方式存在缺陷,治疗方法仍较传统,而侵袭性高和伴有转移的MEC患者临床远期效果较差,且无靶向治疗。在黏液表皮样肿瘤中t(11; 19)染色体常发生易位,CRTC1和MAML2基因融合,产生嵌合蛋白CRTC1-MAML2。CRTC1-MAML2融合基因作为NOTCH和环腺苷酸效应元件结合蛋白(CREB)调控通路上的转录因子,干扰正常细胞周期和分化,促进肿瘤发育,为人类MEC细胞生长和存活创造条件。因此,CRTC1-MAML2被认为与MEC发生存在因果关系,并为MEC的诊断、预后和特定靶向治疗发展开辟了新的途径。 Mucoepidermoid carcinoma( MEC) is an epithelial malignant tumor composed of mucinous cells,intermediate cells and epidermoid cells,which is the most commonly seen type of malignancy in salivary gland. Currently,the carcinogenic mechanism of MEC is still unclear,and the diagnosis method is defective. The treatment method is relatively traditional,while the long-term clinical effect of patients with aggressive and metastatic MEC is poor,and there is no targeted treatment.A considerable number of mucoepidermoid tumors was found t( 11;19) chromosomal translocation,CRTC1 and MAML2 gene fused to produce chimeric protein CRTC1-MAML2. As transcription factors in NOTCH and c AMP-response element binding protein( CREB) regulatory pathways,CRTC1-MAML2 fusion gene interferes with normal cell cycle and differentiation and promotes tumor development,which is necessary for the growth and survival of human MEC cells. Therefore,CRTC1-MAML2 is considered as the carcinogenic cause of MEC,and opens a new way for the diagnosis,prognosis and development of specific targeted therapy of MEC.
作者 王娟 吴发印 徐海丽 WANG Juan;WU Fayin;XU Haili(Depattment of Stomatology,Zhuhai Hospital/the Fifth Affiliated Hospital of Zunyi Medical University,Zhuhai 519100,China)
出处 《医学综述》 2019年第6期1117-1122,共6页 Medical Recapitulate
基金 贵州省科学技术基金(黔科合J字LKZ〔2010〕07号)
关键词 涎腺黏液表皮样癌 CRCT1-MAML2融合基因 靶向治疗 Mucoepidermoid carcinoma of salivary gland CRTC1-MAML2 fusion gene Targeted therapy
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