FUS::DDIT3 Fusion Protein in the Development of Myxoid Liposarcoma and Possible Implications for Therapy

Xutong Hou; Wenjin Shi; Wenxin Luo; Yuwen Luo; Xuelin Huang; Jing Li; Ning Ji; Qianming Chen

doi:10.3390/biom14101297

FUS::DDIT3 Fusion Protein in the Development of Myxoid Liposarcoma and Possible Implications for Therapy

Biomolecules. 2024 Oct 14;14(10):1297. doi: 10.3390/biom14101297.

Authors

Xutong Hou¹, Wenjin Shi¹, Wenxin Luo¹, Yuwen Luo¹, Xuelin Huang¹, Jing Li¹, Ning Ji¹, Qianming Chen¹

Affiliation

¹ 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.

Abstract

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.

Keywords: FUS::DDIT3 fusion protein; chromosomal translocation; myxoid liposarcoma.

Publication types

Review

MeSH terms

Gene Expression Regulation, Neoplastic / drug effects
Humans
Liposarcoma, Myxoid* / drug therapy
Liposarcoma, Myxoid* / genetics
Liposarcoma, Myxoid* / metabolism
Liposarcoma, Myxoid* / pathology
Oncogene Proteins, Fusion* / genetics
Oncogene Proteins, Fusion* / metabolism
RNA-Binding Protein FUS* / genetics
RNA-Binding Protein FUS* / metabolism

Substances

FUS protein, human
FUS-DDIT3 fusion protein, human
Oncogene Proteins, Fusion
RNA-Binding Protein FUS

Grants and funding

This work was supported by the Key Project of the Science and Technology Department of Sichuan Province (2022YFS0003), the CAMS Innovation Fund for Medical Sciences (CIFMS, 2019-I2M-5-004).