State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, Research Unit of Oral Carcinogenesis and Management, Chinese Academy of Medical Sciences, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.
Biomolecules. 2024 Oct 14;14(10):1297. doi: 10.3390/biom14101297.
The FUS::DDIT3 fusion protein, formed by the chromosomal translocation t (12;16) (q13;p11), is found in over 90% of myxoid liposarcoma (MLS) cases and is a crucial protein in its development. Many studies have explored the role of FUS::DDIT3 in MLS, and the prevailing view is that FUS::DDIT3 inhibits adipocyte differentiation and promotes MLS growth and invasive migration by functioning as an aberrant transcription factor that affects gene expression and regulates its downstream molecules. As fusion proteins are gradually showing their potential as targets for precision cancer therapy, FUS::DDIT3 has also been investigated as a therapeutic target. Drugs that target FUS::DDIT3 and its downstream molecules for treating MLS are widely utilized in both clinical practice and experimental studies, and some of them have demonstrated promising results. This article reviews the findings of relevant research, providing an overview of the oncogenic mechanisms of the FUS::DDIT3 fusion protein in MLS, as well as recent advancements in its therapy.
:DDIT3 融合蛋白由染色体易位 t(12;16)(q13;p11)形成，存在于超过 90%的黏液样脂肪肉瘤 (MLS) 病例中，是其发展的关键蛋白。许多研究探索了 FUS::DDIT3 在 MLS 中的作用，目前的观点是，FUS::DDIT3 通过作为一种异常转录因子发挥作用，影响基因表达并调节其下游分子，从而抑制脂肪细胞分化并促进 MLS 的生长和侵袭迁移。随着融合蛋白逐渐显示出作为精准癌症治疗靶点的潜力，FUS::DDIT3 也被作为治疗靶点进行了研究。针对 FUS::DDIT3 及其下游分子治疗 MLS 的药物在临床实践和实验研究中都得到了广泛应用，其中一些已经显示出有希望的结果。本文综述了相关研究的发现，概述了 FUS::DDIT3 融合蛋白在 MLS 中的致癌机制，以及其治疗的最新进展。
