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FUS-DDIT3 Fusion Protein Expression

MedGen UID:
309123
•Concept ID:
C1517100
•
Cell or Molecular Dysfunction
Synonyms: FUS-CHOP Fusion Protein Expression; FUS/CHOP Fusion Protein Expression; FUS/DDIT3 Fusion Protein Expression

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFUS-DDIT3 Fusion Protein Expression

Recent clinical studies

Etiology

FET family fusion oncoproteins target the SWI/SNF chromatin remodeling complex.
Lindén M, Thomsen C, Grundevik P, Jonasson E, Andersson D, Runnberg R, Dolatabadi S, Vannas C, Luna Santamarίa M, Fagman H, Ståhlberg A, Åman P
EMBO Rep 2019 May;20(5) Epub 2019 Apr 8 doi: 10.15252/embr.201845766. PMID: 30962207Free PMC Article
Bromodomain and extraterminal proteins foster the core transcriptional regulatory programs and confer vulnerability in liposarcoma.
Chen Y, Xu L, Mayakonda A, Huang ML, Kanojia D, Tan TZ, Dakle P, Lin RY, Ke XY, Said JW, Chen J, Gery S, Ding LW, Jiang YY, Pang A, Puhaindran ME, Goh BC, Koeffler HP
Nat Commun 2019 Mar 22;10(1):1353. doi: 10.1038/s41467-019-09257-z. PMID: 30903020Free PMC Article
Phosphatidylinositol-3-kinase (PI3K)/Akt Signaling is Functionally Essential in Myxoid Liposarcoma.
Trautmann M, Cyra M, Isfort I, Jeiler B, Krüger A, Grünewald I, Steinestel K, Altvater B, Rossig C, Hafner S, Simmet T, Becker J, Åman P, Wardelmann E, Huss S, Hartmann W
Mol Cancer Ther 2019 Apr;18(4):834-844. Epub 2019 Feb 20 doi: 10.1158/1535-7163.MCT-18-0763. PMID: 30787173
FGFR2 is overexpressed in myxoid liposarcoma and inhibition of FGFR signaling impairs tumor growth in vitro.
Künstlinger H, Fassunke J, Schildhaus HU, Brors B, Heydt C, Ihle MA, Mechtersheimer G, Wardelmann E, Büttner R, Merkelbach-Bruse S
Oncotarget 2015 Aug 21;6(24):20215-30. doi: 10.18632/oncotarget.4046. PMID: 26036639Free PMC Article

Diagnosis

Spindle cell liposarcoma with a TRIO-TERT fusion transcript.
Suster DI, Deshpande V, Chebib I, Taylor MS, Mullen J, Bredella MA, Nielsen GP
Virchows Arch 2019 Sep;475(3):391-394. Epub 2019 Feb 22 doi: 10.1007/s00428-019-02545-5. PMID: 30793229
FGFR2 is overexpressed in myxoid liposarcoma and inhibition of FGFR signaling impairs tumor growth in vitro.
Künstlinger H, Fassunke J, Schildhaus HU, Brors B, Heydt C, Ihle MA, Mechtersheimer G, Wardelmann E, Büttner R, Merkelbach-Bruse S
Oncotarget 2015 Aug 21;6(24):20215-30. doi: 10.18632/oncotarget.4046. PMID: 26036639Free PMC Article
Deep sequencing in cancer research.
Yoshida K, Sanada M, Ogawa S
Jpn J Clin Oncol 2013 Feb;43(2):110-5. Epub 2012 Dec 5 doi: 10.1093/jjco/hys206. PMID: 23225907

Prognosis

High prevalence of TERT aberrations in myxoid liposarcoma: TERT reactivation may play a crucial role in tumorigenesis.
Kunieda J, Yamashita K, Togashi Y, Baba S, Sakata S, Inamura K, Ae K, Matsumoto S, Machinami R, Kitagawa M, Takeuchi K
Cancer Sci 2022 Mar;113(3):1078-1089. Epub 2022 Jan 11 doi: 10.1111/cas.15256. PMID: 34971481Free PMC Article
Molecular signatures of tumor progression in myxoid liposarcoma identified by N-glycan mass spectrometry imaging.
Heijs B, Holst-Bernal S, de Graaff MA, Briaire-de Bruijn IH, Rodriguez-Girondo M, van de Sande MAJ, Wuhrer M, McDonnell LA, Bovée JVMG
Lab Invest 2020 Sep;100(9):1252-1261. Epub 2020 Apr 27 doi: 10.1038/s41374-020-0435-2. PMID: 32341520
FGFR2 is overexpressed in myxoid liposarcoma and inhibition of FGFR signaling impairs tumor growth in vitro.
Künstlinger H, Fassunke J, Schildhaus HU, Brors B, Heydt C, Ihle MA, Mechtersheimer G, Wardelmann E, Büttner R, Merkelbach-Bruse S
Oncotarget 2015 Aug 21;6(24):20215-30. doi: 10.18632/oncotarget.4046. PMID: 26036639Free PMC Article
Relevance of translocation type in myxoid liposarcoma and identification of a novel EWSR1-DDIT3 fusion.
Bode-Lesniewska B, Frigerio S, Exner U, Abdou MT, Moch H, Zimmermann DR
Genes Chromosomes Cancer 2007 Nov;46(11):961-71. doi: 10.1002/gcc.20478. PMID: 17647282

Clinical prediction guides

High prevalence of TERT aberrations in myxoid liposarcoma: TERT reactivation may play a crucial role in tumorigenesis.
Kunieda J, Yamashita K, Togashi Y, Baba S, Sakata S, Inamura K, Ae K, Matsumoto S, Machinami R, Kitagawa M, Takeuchi K
Cancer Sci 2022 Mar;113(3):1078-1089. Epub 2022 Jan 11 doi: 10.1111/cas.15256. PMID: 34971481Free PMC Article
Myxoid liposarcoma: it's a hippo's world.
Regina C, Hettmer S
EMBO Mol Med 2019 May;11(5) doi: 10.15252/emmm.201910470. PMID: 30979710Free PMC Article
Normal and functional TP53 in genetically stable myxoid/round cell liposarcoma.
Ståhlberg A, Kåbjörn Gustafsson C, Engtröm K, Thomsen C, Dolatabadi S, Jonasson E, Li CY, Ruff D, Chen SM, Åman P
PLoS One 2014;9(11):e113110. Epub 2014 Nov 13 doi: 10.1371/journal.pone.0113110. PMID: 25393000Free PMC Article
Nuclear expression of FLT1 and its ligand PGF in FUS-DDIT3 carrying myxoid liposarcomas suggests the existence of an intracrine signaling loop.
Andersson MK, Göransson M, Olofsson A, Andersson C, Aman P
BMC Cancer 2010 Jun 1;10:249. doi: 10.1186/1471-2407-10-249. PMID: 20515481Free PMC Article
DDIT3/CHOP and the sarcoma fusion oncoprotein FUS-DDIT3/TLS-CHOP bind cyclin-dependent kinase 2.
Bento C, Andersson MK, Aman P
BMC Cell Biol 2009 Dec 17;10:89. doi: 10.1186/1471-2121-10-89. PMID: 20017906Free PMC Article

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